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Unexplained young onset end-stage renal disease - additional genes v1.3 SIX5 Arina Puzriakova Classified gene: SIX5 as Green List (high evidence)
Unexplained young onset end-stage renal disease - additional genes v1.3 SIX5 Arina Puzriakova Added comment: Comment on list classification: As the evidence for the association of SIX5 with branchio-oto-renal syndrome is disputed, this gene should be considered for demotion to red rating in the next GMS update.
Unexplained young onset end-stage renal disease - additional genes v1.3 SIX5 Arina Puzriakova Gene: six5 has been classified as Green List (High Evidence).
Unexplained young onset end-stage renal disease - additional genes v1.2 SIX5 Arina Puzriakova Tag Q3_25_expert_review tag was added to gene: SIX5.
Tag disputed tag was added to gene: SIX5.
Tag Q3_25_demote_red tag was added to gene: SIX5.
Unexplained young onset end-stage renal disease - additional genes v1.2 SIX5 Arina Puzriakova edited their review of gene: SIX5: Changed rating: RED
Unexplained young onset end-stage renal disease - additional genes v1.2 SIX5 Arina Puzriakova commented on gene: SIX5
Unexplained young onset end-stage renal disease - additional genes v1.2 Achchuthan Shanmugasundram Panel version 1.1 has been signed off on 2025-04-30
Unexplained young onset end-stage renal disease - additional genes v1.1 Achchuthan Shanmugasundram Panel version 1.0 has been signed off on 2024-10-30
Unexplained young onset end-stage renal disease - additional genes v1.0 Achchuthan Shanmugasundram promoted panel to version 1.0
Unexplained young onset end-stage renal disease - additional genes v0.131 Achchuthan Shanmugasundram Panel types changed to Component Of Super Panel; GMS signed-off
Unexplained young onset end-stage renal disease - additional genes v0.129 Achchuthan Shanmugasundram Panel status changed from internal to public
Unexplained young onset end-stage renal disease - additional genes v0.128 Achchuthan Shanmugasundram Panel status changed from public to internal
Unexplained young onset end-stage renal disease - additional genes v0.127 Achchuthan Shanmugasundram Panel status changed from internal to public
Unexplained young onset end-stage renal disease - additional genes v0.126 PRDM15 Arina Puzriakova Tag gene-checked tag was added to gene: PRDM15.
Unexplained young onset end-stage renal disease - additional genes v0.126 Achchuthan Shanmugasundram Panel status changed from public to internal
Unexplained young onset end-stage renal disease - additional genes v0.125 Achchuthan Shanmugasundram Panel status changed from internal to public
Unexplained young onset end-stage renal disease - additional genes v0.124 Achchuthan Shanmugasundram Panel types changed to Component Of Super Panel
Unexplained young onset end-stage renal disease - additional genes v0.122 SOX17 Achchuthan Shanmugasundram Phenotypes for gene: SOX17 were changed from Vesicoureteral reflux 3, 613674 to Vesicoureteral reflux 3, OMIM:613674
Unexplained young onset end-stage renal disease - additional genes v0.121 SIX1 Achchuthan Shanmugasundram Phenotypes for gene: SIX1 were changed from Branchiootorenal Spectrum Disorders to Branchiootic syndrome 3, OMIM:608389; Deafness, autosomal dominant 23, OMIM:605192
Unexplained young onset end-stage renal disease - additional genes v0.120 ROBO2 Achchuthan Shanmugasundram Phenotypes for gene: ROBO2 were changed from Vesicoureteral reflux 2, 610878; Vesicoureteral Reflux to Vesicoureteral reflux 2, OMIM:610878
Unexplained young onset end-stage renal disease - additional genes v0.119 MYH11 Achchuthan Shanmugasundram Phenotypes for gene: MYH11 were changed from Megacystis-microcolon-intestinal hypoperistalsis syndrome to Megacystis-microcolon-intestinal hypoperistalsis syndrome 2, OMIM:619351
Unexplained young onset end-stage renal disease - additional genes v0.118 DACT1 Achchuthan Shanmugasundram Phenotypes for gene: DACT1 were changed from TBS2; ?Townes-Brocks syndrome 2,617466 to Townes-Brocks syndrome 2, OMIM:617466
Unexplained young onset end-stage renal disease - additional genes v0.117 DACT1 Achchuthan Shanmugasundram Publications for gene: DACT1 were set to 19701191; 28054444; 22610794
Unexplained young onset end-stage renal disease - additional genes v0.116 COX10 Achchuthan Shanmugasundram Phenotypes for gene: COX10 were changed from Encephalopathy, progressive mitochondrial, with proximal renal tubulopathy due tocytochrome c oxidase deficiency; Mitochondrial complex IV deficiency, nuclear type 3, OMIM:619046 to Mitochondrial complex IV deficiency, nuclear type 3, OMIM:619046
Unexplained young onset end-stage renal disease - additional genes v0.115 CHD1L Achchuthan Shanmugasundram Phenotypes for gene: CHD1L were changed from Renal or urinary tract malformation (CAKUT); ORPHA93545 to congenital anomaly of kidney and urinary tract, MONDO:0019719
Unexplained young onset end-stage renal disease - additional genes v0.114 CHD1L Achchuthan Shanmugasundram Publications for gene: CHD1L were set to 24429398; 22146311
Unexplained young onset end-stage renal disease - additional genes v0.113 BMP4 Achchuthan Shanmugasundram Phenotypes for gene: BMP4 were changed from to Microphthalmia, syndromic 6, OMIM:607932
Unexplained young onset end-stage renal disease - additional genes v0.112 BICC1 Achchuthan Shanmugasundram Phenotypes for gene: BICC1 were changed from {Renal dysplasia, cystic, susceptibility to}, 601331 to {Renal dysplasia, cystic, susceptibility to}, OMIM:601331
Unexplained young onset end-stage renal disease - additional genes v0.111 ACTA2 Achchuthan Shanmugasundram Phenotypes for gene: ACTA2 were changed from Multi system smooth muscle dysfunction to Smooth muscle dysfunction syndrome, OMIM:613834
Unexplained young onset end-stage renal disease - additional genes v0.110 WDR72 Achchuthan Shanmugasundram Phenotypes for gene: WDR72 were changed from distal renal tubular acidosis, MONDO:0015827; hereditary distal renal tubular acidosis; Amelogenesis imperfecta, type IIA3, OMIM:613211; amelogenesis imperfecta hypomaturation type 2A3, MONDO:0013181 to distal renal tubular acidosis, MONDO:0015827; Amelogenesis imperfecta, type IIA3, OMIM:613211; amelogenesis imperfecta hypomaturation type 2A3, MONDO:0013181
Unexplained young onset end-stage renal disease - additional genes v0.109 WDR72 Achchuthan Shanmugasundram Publications for gene: WDR72 were set to 30779877; 33033857; 30028003; 31959358
Unexplained young onset end-stage renal disease - additional genes v0.108 TRAP1 Achchuthan Shanmugasundram Phenotypes for gene: TRAP1 were changed from CAKUT; VACTERL 192350 to CAKUT; VATER/VACTERL ASSOCIATION, OMIM:192350
Unexplained young onset end-stage renal disease - additional genes v0.107 TBX18 Achchuthan Shanmugasundram Phenotypes for gene: TBX18 were changed from Congenital anomalies of kidney and urinary tract 2 143400 to Congenital anomalies of kidney and urinary tract 2, OMIM:143400
Unexplained young onset end-stage renal disease - additional genes v0.106 TBX18 Achchuthan Shanmugasundram Publications for gene: TBX18 were set to
Unexplained young onset end-stage renal disease - additional genes v0.105 SIX5 Achchuthan Shanmugasundram Phenotypes for gene: SIX5 were changed from Branchiootorenal syndrome 2, 610896 to Branchiootorenal syndrome 2, OMIM:610896
Unexplained young onset end-stage renal disease - additional genes v0.104 SALL1 Achchuthan Shanmugasundram Phenotypes for gene: SALL1 were changed from Townes-Brocks branchiootorenal-like syndrome, 107480; Townes-Brocks syndrome, 107480; imperforate anus, ear abnormalities, thumb abnormalities to Townes-Brocks branchiootorenal-like syndrome, OMIM:107480; Townes-Brocks syndrome 1, OMIM:107480
Unexplained young onset end-stage renal disease - additional genes v0.103 RRM2B Achchuthan Shanmugasundram Phenotypes for gene: RRM2B were changed from Mitochondrial DNA depletion syndrome 8A (encephalomyopathic type with renal tubulopathy) 612075; Mitochondrial DNA depletion syndrome 8B (MNGIE type) 612075; Mitochondrial DNA depletion syndrome 8A (encephalomyopathic,renal tubulopathy), 612075 to Mitochondrial DNA depletion syndrome 8A (encephalomyopathic type with renal tubulopathy), OMIM:612075; Mitochondrial DNA depletion syndrome 8B (MNGIE type), OMIM:612075; Rod-cone dystrophy, sensorineural deafness, and Fanconi-type renal dysfunction, OMIM:268315
Unexplained young onset end-stage renal disease - additional genes v0.102 RMND1 Achchuthan Shanmugasundram Publications for gene: RMND1 were set to 32911714; 31889854; 31568715
Unexplained young onset end-stage renal disease - additional genes v0.101 RET Achchuthan Shanmugasundram Deleted their comment
Unexplained young onset end-stage renal disease - additional genes v0.101 RET Achchuthan Shanmugasundram Added comment: Comment on phenotypes: 191830
Unexplained young onset end-stage renal disease - additional genes v0.101 RET Achchuthan Shanmugasundram Phenotypes for gene: RET were changed from {Hirschsprung disease, susceptibility to, 1}, 142623; Multiple endocrine neoplasia IIA, 171400; Renal Adysplasia; Pheochromocytoma, 171300; Renal agenesis, 191830; Central hypoventilation syndrome, congenital, 209880; Multiple endocrine neoplasia IIB, 162300; Medullary thyroid carcinoma, 155240 to {Hirschsprung disease, susceptibility to, 1}, OMIM:142623; Multiple endocrine neoplasia IIA, OMIM:171400; Multiple endocrine neoplasia IIB, OMIM:162300; Pheochromocytoma, OMIM:171300
Unexplained young onset end-stage renal disease - additional genes v0.100 PBX1 Achchuthan Shanmugasundram Phenotypes for gene: PBX1 were changed from CAKUT to Congenital anomalies of kidney and urinary tract syndrome with or without hearing loss, abnormal ears, or developmental delay, OMIM:617641
Unexplained young onset end-stage renal disease - additional genes v0.99 LRIG2 Achchuthan Shanmugasundram Phenotypes for gene: LRIG2 were changed from Congenital bladder disease: dyssynergic, high pressure bladder.; Urofacial syndrome; Urofacial syndrome 2 615112 to Urofacial syndrome 2, OMIM:615112; Congenital bladder disease: dyssynergic, high pressure bladder
Unexplained young onset end-stage renal disease - additional genes v0.98 LRIG2 Achchuthan Shanmugasundram Publications for gene: LRIG2 were set to Stuart HM, Roberts NA, Bergu B, Daly SB, Urquhart JE, Bhaskar S, Dickerson J, Mermerkaya M, Silay MS, Lewis MA, Olondriz BO, Gener B, Beetz C, Varga RE, G lp nar O, S er E, Yal nkaya F, G c k A, Yue WW, Erdogan F, Berry A, Hanley NA, McKenzie EA, Hilton EN, Woolf AS, Newman WG. LRIG2 mutations cause urofacial syndrome. Am J Hum Genet 92:259-264, 2013.
Unexplained young onset end-stage renal disease - additional genes v0.97 KYNU Achchuthan Shanmugasundram Phenotypes for gene: KYNU were changed from Hydroxykynureninuria (Disorders of histidine, tryptophan or lysine metabolism); VACTERL-like phenotype; ?Hydroxykynureninuria, 236800; multiple congenital malformations to Vertebral, cardiac, renal, and limb defects syndrome 2, OMIM:617661
Unexplained young onset end-stage renal disease - additional genes v0.96 KYNU Achchuthan Shanmugasundram Publications for gene: KYNU were set to 28792876; 27604308; 17334708
Unexplained young onset end-stage renal disease - additional genes v0.95 ITGA8 Achchuthan Shanmugasundram Phenotypes for gene: ITGA8 were changed from Renal hypodysplasia/aplasia 1, 191830 to Renal hypodysplasia/aplasia 1, OMIM:191830
Unexplained young onset end-stage renal disease - additional genes v0.94 ISCA-37401-Loss Achchuthan Shanmugasundram Phenotypes for Region: ISCA-37401-Loss were changed from 194072; Wilms tumor, aniridia, genitourinary anomalies and mental retardation syndrome to Wilms tumor, aniridia, genitourinary anomalies and impaired intellectual development syndrome, OMIM:194072
Unexplained young onset end-stage renal disease - additional genes v0.93 HPSE2 Achchuthan Shanmugasundram Phenotypes for gene: HPSE2 were changed from Urofacial Syndrome; Urofacial syndrome 1 236730; Congenital bladder disease: dyssynergic, high pressure bladder to Urofacial syndrome 1, OMIM:236730; Congenital bladder disease: dyssynergic, high pressure bladder
Unexplained young onset end-stage renal disease - additional genes v0.92 HAAO Achchuthan Shanmugasundram Phenotypes for gene: HAAO were changed from Multiple congenital malformations; VACTERL-like phenotype to Vertebral, cardiac, renal, and limb defects syndrome 1, OMIM:617660
Unexplained young onset end-stage renal disease - additional genes v0.91 HAAO Achchuthan Shanmugasundram Publications for gene: HAAO were set to 28792876; 27604308; 17334708
Unexplained young onset end-stage renal disease - additional genes v0.90 GRIP1 Achchuthan Shanmugasundram Phenotypes for gene: GRIP1 were changed from Fraser syndrome; isolated CAKUT; Fraser syndrome 219000 to Fraser syndrome 3, OMIM:617667
Unexplained young onset end-stage renal disease - additional genes v0.89 GATA3 Achchuthan Shanmugasundram Phenotypes for gene: GATA3 were changed from Hypoparathyroidism, Sensorineural Deafness, and Renal Disease; Hypoparathyroidism, sensorineural deafness, and renal dysplasia, 146255 to Hypoparathyroidism, sensorineural deafness, and renal dysplasia, OMIM:146255
Unexplained young onset end-stage renal disease - additional genes v0.88 FREM2 Achchuthan Shanmugasundram Phenotypes for gene: FREM2 were changed from Fraser syndrome; Fraser syndrome 219000 to Fraser syndrome 2, OMIM:617666
Unexplained young onset end-stage renal disease - additional genes v0.87 FREM1 Achchuthan Shanmugasundram Phenotypes for gene: FREM1 were changed from Bifid nose with or without anorectal and renal anomalies, 608980 to Bifid nose with or without anorectal and renal anomalies, OMIM:608980
Unexplained young onset end-stage renal disease - additional genes v0.86 FREM1 Achchuthan Shanmugasundram Publications for gene: FREM1 were set to PMID: 24700879
Unexplained young onset end-stage renal disease - additional genes v0.85 FREM1 Achchuthan Shanmugasundram Publications for gene: FREM1 were set to PMID: 24700879
Unexplained young onset end-stage renal disease - additional genes v0.84 FRAS1 Achchuthan Shanmugasundram Phenotypes for gene: FRAS1 were changed from Fraser syndrome; Fraser syndrome 219000 to Fraser syndrome 1, OMIM:219000
Unexplained young onset end-stage renal disease - additional genes v0.83 FAN1 Achchuthan Shanmugasundram Phenotypes for gene: FAN1 were changed from chronic kidney disease; karyomegalic interstitial nephritis, MONDO:0013898; interstitial nephritis; Interstitial nephritis, karyomegalic, OMIM:614817 to Interstitial nephritis, karyomegalic, OMIM:614817; karyomegalic interstitial nephritis, MONDO:0013898; chronic kidney disease
Unexplained young onset end-stage renal disease - additional genes v0.82 EYA1 Achchuthan Shanmugasundram Phenotypes for gene: EYA1 were changed from Anterior segment anomalies with or without cataract, 113650; Branchiootorenal Spectrum Disorders; Otofaciocervical syndrome, 166780; Branchiootorenal syndrome 1, with or without cataracts; Branchiootorenal syndrome 1, with or without cataracts, 113650; Branchiootic syndrome 1, 602588 to Branchiootorenal syndrome 1, with or without cataracts, OMIM:113650
Unexplained young onset end-stage renal disease - additional genes v0.81 DSTYK Achchuthan Shanmugasundram Phenotypes for gene: DSTYK were changed from vesicoureteric reflux; CONGENITAL ANOMALIES OF KIDNEY AND URINARY TRACT, CAKUT1; Renal hypodysplasia; {Congenital anomalies of kidney and urinary tract, susceptibility to}, 610805; ureteropelvic junction obstruction; {Congenital anomalies of kidney and urinary tract, susceptibility to} to Congenital anomalies of kidney and urinary tract 1, OMIM:610805
Unexplained young onset end-stage renal disease - additional genes v0.80 CHRM3 Achchuthan Shanmugasundram Phenotypes for gene: CHRM3 were changed from Megacystis; Urinary Bladder Disease; Prune belly syndrome, OMIM:100100 to Prune belly syndrome, OMIM:100100; Megacystis; Urinary Bladder Disease
Unexplained young onset end-stage renal disease - additional genes v0.79 CHRM3 Achchuthan Shanmugasundram Publications for gene: CHRM3 were set to 31441039; 22077972; 10944224
Unexplained young onset end-stage renal disease - additional genes v0.78 CHD7 Achchuthan Shanmugasundram Phenotypes for gene: CHD7 were changed from CHARGE syndrome 214800 to CHARGE syndrome, OMIM:214800
Unexplained young onset end-stage renal disease - additional genes v0.77 C3 Achchuthan Shanmugasundram Phenotypes for gene: C3 were changed from C3 deficiency 613779 AR; {Hemolytic uremic syndrome, atypical, susceptibility to, 5} 612925 AD to C3 deficiency, OMIM:613779; {Hemolytic uremic syndrome, atypical, susceptibility to, 5}, OMIM:612925
Unexplained young onset end-stage renal disease - additional genes v0.76 BNC2 Achchuthan Shanmugasundram Phenotypes for gene: BNC2 were changed from Congenital lower urinary-tract obstruction; Lower urinary tract obstruction, congenital, 618612; Posterior urethral valves; PUV to Lower urinary tract obstruction, congenital, OMIM:618612
Unexplained young onset end-stage renal disease - additional genes v0.75 ARMC9 Achchuthan Shanmugasundram Phenotypes for gene: ARMC9 were changed from Joubert syndrome 30, 617622 to Joubert syndrome 30, OMIM:617622
Unexplained young onset end-stage renal disease - additional genes v0.74 ANOS1 Achchuthan Shanmugasundram Phenotypes for gene: ANOS1 were changed from Kallman syndrome; Hypogonadotropic hypogonadism 1 with or without anosmia (Kallmann syndrome 1) to Hypogonadotropic hypogonadism 1 with or without anosmia (Kallmann syndrome 1), OMIM:308700
Unexplained young onset end-stage renal disease - additional genes v0.73 ACTG2 Achchuthan Shanmugasundram Publications for gene: ACTG2 were set to PMID: 25998219
Unexplained young onset end-stage renal disease - additional genes v0.72 AGTR1 Achchuthan Shanmugasundram Phenotypes for gene: AGTR1 were changed from Hypertension, essential, 145500; Renal Tubular Dysgenesis; Renal tubular dysgenesis, 267430 to Renal tubular dysgenesis, OMIM:267430
Unexplained young onset end-stage renal disease - additional genes v0.71 AGT Achchuthan Shanmugasundram Phenotypes for gene: AGT were changed from Renal tubular dysgenesis, 267430; Renal Tubular Dysgenesis; {Hypertension, essential, susceptibility to}, 145500{Preeclampsia, susceptibility to}Renal tubular dysgenesis, 267430 to Renal tubular dysgenesis, OMIM:267430
Unexplained young onset end-stage renal disease - additional genes v0.70 ACTG2 Achchuthan Shanmugasundram Phenotypes for gene: ACTG2 were changed from Berdon syndrome; visceral myopathy; Megacystis-microcolon intestinal hypoperistalsis syndrome; Visceral myopathy (Megacystis-microcolon intestinal hypoperistalsis syndrome, Berdon syndrome) 155310 to Megacystis-microcolon-intestinal hypoperistalsis syndrome 5, OMIM:619431; Visceral myopathy 1, OMIM:155310; Berdon syndrome
Unexplained young onset end-stage renal disease - additional genes v0.69 ACE Achchuthan Shanmugasundram Phenotypes for gene: ACE were changed from {Myocardial infarction, susceptibility to}{Alzheimer disease, susceptibility to}, 104300{Microvascular complications of diabetes 3}, 612624[Angiotensin I-converting enzyme, benign serum increase]{SARS, progression of}Renal tubular; Renal Tubular Dysgenesis; Renal Tubular Dysgenesis 267430 to Renal tubular dysgenesis, OMIM:267430
Unexplained young onset end-stage renal disease - additional genes v0.68 ACE Achchuthan Shanmugasundram edited their review of gene: ACE: Changed phenotypes to: Renal tubular dysgenesis, OMIM:267430
Unexplained young onset end-stage renal disease - additional genes v0.68 ISCA-37401-Loss Achchuthan Shanmugasundram Triplosensitivity Score for ISCA-37401-Loss was changed from None to .
Unexplained young onset end-stage renal disease - additional genes v0.68 WDR72 Achchuthan Shanmugasundram Added phenotypes distal renal tubular acidosis, MONDO:0015827; hereditary distal renal tubular acidosis; Amelogenesis imperfecta, type IIA3, OMIM:613211; amelogenesis imperfecta hypomaturation type 2A3, MONDO:0013181 for gene: WDR72
Publications for gene: WDR72 were updated from 30028003; 30779877; 31959358; 33033857 to 30779877; 33033857; 30028003; 31959358
Unexplained young onset end-stage renal disease - additional genes v0.68 UPK3A Achchuthan Shanmugasundram Added phenotypes Renal Adysplasia for gene: UPK3A
Unexplained young onset end-stage renal disease - additional genes v0.68 TRAP1 Achchuthan Shanmugasundram Added phenotypes CAKUT; VACTERL 192350 for gene: TRAP1
Unexplained young onset end-stage renal disease - additional genes v0.68 TBX18 Achchuthan Shanmugasundram Added phenotypes Congenital anomalies of kidney and urinary tract 2 143400 for gene: TBX18
Unexplained young onset end-stage renal disease - additional genes v0.68 SOX17 Achchuthan Shanmugasundram Added phenotypes Vesicoureteral reflux 3, 613674 for gene: SOX17
Unexplained young onset end-stage renal disease - additional genes v0.68 SIX5 Achchuthan Shanmugasundram Added phenotypes Branchiootorenal syndrome 2, 610896 for gene: SIX5
Unexplained young onset end-stage renal disease - additional genes v0.68 SIX1 Achchuthan Shanmugasundram Added phenotypes Branchiootorenal Spectrum Disorders for gene: SIX1
Unexplained young onset end-stage renal disease - additional genes v0.68 SALL1 Achchuthan Shanmugasundram Added phenotypes Townes-Brocks branchiootorenal-like syndrome, 107480; Townes-Brocks syndrome, 107480; imperforate anus, ear abnormalities, thumb abnormalities for gene: SALL1
Unexplained young onset end-stage renal disease - additional genes v0.68 RRM2B Achchuthan Shanmugasundram Added phenotypes Mitochondrial DNA depletion syndrome 8A (encephalomyopathic type with renal tubulopathy) 612075; Mitochondrial DNA depletion syndrome 8B (MNGIE type) 612075; Mitochondrial DNA depletion syndrome 8A (encephalomyopathic,renal tubulopathy), 612075 for gene: RRM2B
Unexplained young onset end-stage renal disease - additional genes v0.68 ROBO2 Achchuthan Shanmugasundram Added phenotypes Vesicoureteral reflux 2, 610878; Vesicoureteral Reflux for gene: ROBO2
Unexplained young onset end-stage renal disease - additional genes v0.68 RMND1 Achchuthan Shanmugasundram Added phenotypes Combined oxidative phosphorylation deficiency 11, OMIM:614922 for gene: RMND1
Publications for gene: RMND1 were updated from 31568715; 31889854; 32911714 to 32911714; 31889854; 31568715
Unexplained young onset end-stage renal disease - additional genes v0.68 RET Achchuthan Shanmugasundram Added phenotypes {Hirschsprung disease, susceptibility to, 1}, 142623; Multiple endocrine neoplasia IIA, 171400; Renal Adysplasia; Pheochromocytoma, 171300; Renal agenesis, 191830; Central hypoventilation syndrome, congenital, 209880; Multiple endocrine neoplasia IIB, 162300; Medullary thyroid carcinoma, 155240 for gene: RET
Unexplained young onset end-stage renal disease - additional genes v0.68 PBX1 Achchuthan Shanmugasundram Added phenotypes CAKUT for gene: PBX1
Unexplained young onset end-stage renal disease - additional genes v0.68 MYH11 Achchuthan Shanmugasundram Added phenotypes Megacystis-microcolon-intestinal hypoperistalsis syndrome for gene: MYH11
Unexplained young onset end-stage renal disease - additional genes v0.68 LRIG2 Achchuthan Shanmugasundram Added phenotypes Congenital bladder disease: dyssynergic, high pressure bladder.; Urofacial syndrome; Urofacial syndrome 2 615112 for gene: LRIG2
Unexplained young onset end-stage renal disease - additional genes v0.68 KYNU Achchuthan Shanmugasundram Added phenotypes Hydroxykynureninuria (Disorders of histidine, tryptophan or lysine metabolism); VACTERL-like phenotype; ?Hydroxykynureninuria, 236800; multiple congenital malformations for gene: KYNU
Publications for gene: KYNU were updated from 27604308; 17334708; 28792876 to 28792876; 27604308; 17334708
Unexplained young onset end-stage renal disease - additional genes v0.68 ITGA8 Achchuthan Shanmugasundram Added phenotypes Renal hypodysplasia/aplasia 1, 191830 for gene: ITGA8
Unexplained young onset end-stage renal disease - additional genes v0.68 HPSE2 Achchuthan Shanmugasundram Added phenotypes Urofacial Syndrome; Urofacial syndrome 1 236730; Congenital bladder disease: dyssynergic, high pressure bladder for gene: HPSE2
Publications for gene: HPSE2 were updated from 20560210; 20560209 to 20560209; 20560210
Unexplained young onset end-stage renal disease - additional genes v0.68 HAAO Achchuthan Shanmugasundram Added phenotypes Multiple congenital malformations; VACTERL-like phenotype for gene: HAAO
Publications for gene: HAAO were updated from 27604308; 17334708; 28792876 to 28792876; 27604308; 17334708
Unexplained young onset end-stage renal disease - additional genes v0.68 GRIP1 Achchuthan Shanmugasundram Added phenotypes Fraser syndrome; isolated CAKUT; Fraser syndrome 219000 for gene: GRIP1
Publications for gene: GRIP1 were updated from 24700879; 14730302; 24357607; 22510445 to 14730302; 24357607; 24700879; 22510445
Unexplained young onset end-stage renal disease - additional genes v0.68 GLI3 Achchuthan Shanmugasundram Added phenotypes Pallister-Hall syndrome, OMIM:146510 for gene: GLI3
Unexplained young onset end-stage renal disease - additional genes v0.68 GATA3 Achchuthan Shanmugasundram Added phenotypes Hypoparathyroidism, Sensorineural Deafness, and Renal Disease; Hypoparathyroidism, sensorineural deafness, and renal dysplasia, 146255 for gene: GATA3
Unexplained young onset end-stage renal disease - additional genes v0.68 FREM2 Achchuthan Shanmugasundram Added phenotypes Fraser syndrome; Fraser syndrome 219000 for gene: FREM2
Unexplained young onset end-stage renal disease - additional genes v0.68 FREM1 Achchuthan Shanmugasundram Added phenotypes Bifid nose with or without anorectal and renal anomalies, 608980 for gene: FREM1
Unexplained young onset end-stage renal disease - additional genes v0.68 FRAS1 Achchuthan Shanmugasundram Added phenotypes Fraser syndrome; Fraser syndrome 219000 for gene: FRAS1
Unexplained young onset end-stage renal disease - additional genes v0.68 FAN1 Achchuthan Shanmugasundram Added phenotypes chronic kidney disease; karyomegalic interstitial nephritis, MONDO:0013898; interstitial nephritis; Interstitial nephritis, karyomegalic, OMIM:614817 for gene: FAN1
Unexplained young onset end-stage renal disease - additional genes v0.68 EYA1 Achchuthan Shanmugasundram Added phenotypes Anterior segment anomalies with or without cataract, 113650; Branchiootorenal Spectrum Disorders; Otofaciocervical syndrome, 166780; Branchiootorenal syndrome 1, with or without cataracts; Branchiootorenal syndrome 1, with or without cataracts, 113650; Branchiootic syndrome 1, 602588 for gene: EYA1
Unexplained young onset end-stage renal disease - additional genes v0.68 DSTYK Achchuthan Shanmugasundram Added phenotypes vesicoureteric reflux; CONGENITAL ANOMALIES OF KIDNEY AND URINARY TRACT, CAKUT1; Renal hypodysplasia; {Congenital anomalies of kidney and urinary tract, susceptibility to}, 610805; ureteropelvic junction obstruction; {Congenital anomalies of kidney and urinary tract, susceptibility to} for gene: DSTYK
Unexplained young onset end-stage renal disease - additional genes v0.68 DACT1 Achchuthan Shanmugasundram Added phenotypes TBS2; ?Townes-Brocks syndrome 2,617466 for gene: DACT1
Publications for gene: DACT1 were updated from 28054444; 19701191; 22610794 to 19701191; 28054444; 22610794
Unexplained young onset end-stage renal disease - additional genes v0.68 COX10 Achchuthan Shanmugasundram Added phenotypes Encephalopathy, progressive mitochondrial, with proximal renal tubulopathy due tocytochrome c oxidase deficiency; Mitochondrial complex IV deficiency, nuclear type 3, OMIM:619046 for gene: COX10
Unexplained young onset end-stage renal disease - additional genes v0.68 CHRM3 Achchuthan Shanmugasundram Added phenotypes Megacystis; Urinary Bladder Disease; Prune belly syndrome, OMIM:100100 for gene: CHRM3
Publications for gene: CHRM3 were updated from 10944224; 22077972; 31441039 to 31441039; 22077972; 10944224
Unexplained young onset end-stage renal disease - additional genes v0.68 CHD7 Achchuthan Shanmugasundram Added phenotypes CHARGE syndrome 214800 for gene: CHD7
Unexplained young onset end-stage renal disease - additional genes v0.68 CHD1L Achchuthan Shanmugasundram Added phenotypes Renal or urinary tract malformation (CAKUT); ORPHA93545 for gene: CHD1L
Unexplained young onset end-stage renal disease - additional genes v0.68 BNC2 Achchuthan Shanmugasundram Added phenotypes Congenital lower urinary-tract obstruction; Lower urinary tract obstruction, congenital, 618612; Posterior urethral valves; PUV for gene: BNC2
Unexplained young onset end-stage renal disease - additional genes v0.68 BICC1 Achchuthan Shanmugasundram Added phenotypes {Renal dysplasia, cystic, susceptibility to}, 601331 for gene: BICC1
Unexplained young onset end-stage renal disease - additional genes v0.68 ARMC9 Achchuthan Shanmugasundram Added phenotypes Joubert syndrome 30, 617622 for gene: ARMC9
Unexplained young onset end-stage renal disease - additional genes v0.68 ANOS1 Achchuthan Shanmugasundram Added phenotypes Kallman syndrome; Hypogonadotropic hypogonadism 1 with or without anosmia (Kallmann syndrome 1) for gene: ANOS1
Unexplained young onset end-stage renal disease - additional genes v0.68 AGTR1 Achchuthan Shanmugasundram Added phenotypes Hypertension, essential, 145500; Renal Tubular Dysgenesis; Renal tubular dysgenesis, 267430 for gene: AGTR1
Unexplained young onset end-stage renal disease - additional genes v0.68 AGT Achchuthan Shanmugasundram Added phenotypes Renal tubular dysgenesis, 267430; Renal Tubular Dysgenesis; {Hypertension, essential, susceptibility to}, 145500{Preeclampsia, susceptibility to}Renal tubular dysgenesis, 267430 for gene: AGT
Unexplained young onset end-stage renal disease - additional genes v0.68 ACTG2 Achchuthan Shanmugasundram Added phenotypes Berdon syndrome; visceral myopathy; Megacystis-microcolon intestinal hypoperistalsis syndrome; Visceral myopathy (Megacystis-microcolon intestinal hypoperistalsis syndrome, Berdon syndrome) 155310 for gene: ACTG2
Unexplained young onset end-stage renal disease - additional genes v0.68 ACTA2 Achchuthan Shanmugasundram Added phenotypes Multi system smooth muscle dysfunction for gene: ACTA2
Unexplained young onset end-stage renal disease - additional genes v0.68 ACE Achchuthan Shanmugasundram Added phenotypes {Myocardial infarction, susceptibility to}{Alzheimer disease, susceptibility to}, 104300{Microvascular complications of diabetes 3}, 612624[Angiotensin I-converting enzyme, benign serum increase]{SARS, progression of}Renal tubular; Renal Tubular Dysgenesis; Renal Tubular Dysgenesis 267430 for gene: ACE
Unexplained young onset end-stage renal disease - additional genes v0.67 PRDM15 Achchuthan Shanmugasundram Tag Q2_24_promote_green was removed from gene: PRDM15.
Unexplained young onset end-stage renal disease - additional genes v0.67 NOS1AP Achchuthan Shanmugasundram Tag Q1_24_promote_green was removed from gene: NOS1AP.
Unexplained young onset end-stage renal disease - additional genes v0.67 PRDM15 Achchuthan Shanmugasundram commented on gene: PRDM15: The rating of this gene has been updated to Green and the mode of inheritance set to BIALLELIC, autosomal or pseudoautosomal following NHS Genomic Medicine Service approval for this change on R257 Unexplained young onset end-stage renal disease panel.
Unexplained young onset end-stage renal disease - additional genes v0.67 NOS1AP Achchuthan Shanmugasundram commented on gene: NOS1AP: The rating of this gene has been updated to Green and the mode of inheritance set to BIALLELIC, autosomal or pseudoautosomal following NHS Genomic Medicine Service approval for this change on R257 Unexplained young onset end-stage renal disease panel.
Unexplained young onset end-stage renal disease - additional genes v0.66 PRDM15 Achchuthan Shanmugasundram Source Expert Review Green was added to PRDM15.
Source NHS GMS was added to PRDM15.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Unexplained young onset end-stage renal disease - additional genes v0.66 NOS1AP Achchuthan Shanmugasundram Source Expert Review Green was added to NOS1AP.
Source NHS GMS was added to NOS1AP.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Unexplained young onset end-stage renal disease - additional genes v0.65 ISCA-37401-Loss Achchuthan Shanmugasundram reviewed Region: ISCA-37401-Loss: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: None
Unexplained young onset end-stage renal disease - additional genes v0.65 UPK3A Achchuthan Shanmugasundram reviewed gene: UPK3A: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 UPK2 Achchuthan Shanmugasundram reviewed gene: UPK2: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Other
Unexplained young onset end-stage renal disease - additional genes v0.65 TSHZ3 Achchuthan Shanmugasundram reviewed gene: TSHZ3: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 TNXB Achchuthan Shanmugasundram reviewed gene: TNXB: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SPRY1 Achchuthan Shanmugasundram reviewed gene: SPRY1: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SOX17 Achchuthan Shanmugasundram reviewed gene: SOX17: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SMARCA4 Achchuthan Shanmugasundram reviewed gene: SMARCA4: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SLIT2 Achchuthan Shanmugasundram reviewed gene: SLIT2: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SIX1 Achchuthan Shanmugasundram reviewed gene: SIX1: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SHH Achchuthan Shanmugasundram reviewed gene: SHH: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 ROBO2 Achchuthan Shanmugasundram reviewed gene: ROBO2: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 MYH11 Achchuthan Shanmugasundram reviewed gene: MYH11: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 KIT Achchuthan Shanmugasundram reviewed gene: KIT: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 HCN3 Achchuthan Shanmugasundram reviewed gene: HCN3: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 GREM1 Achchuthan Shanmugasundram reviewed gene: GREM1: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 GDNF Achchuthan Shanmugasundram reviewed gene: GDNF: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 FOXC2 Achchuthan Shanmugasundram reviewed gene: FOXC2: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 FOXC1 Achchuthan Shanmugasundram reviewed gene: FOXC1: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 DLG3 Achchuthan Shanmugasundram reviewed gene: DLG3: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 DACT1 Achchuthan Shanmugasundram reviewed gene: DACT1: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 COX10 Achchuthan Shanmugasundram reviewed gene: COX10: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 CHD1L Achchuthan Shanmugasundram reviewed gene: CHD1L: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 BMP4 Achchuthan Shanmugasundram reviewed gene: BMP4: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 BICC1 Achchuthan Shanmugasundram reviewed gene: BICC1: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: Unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 ACTA2 Achchuthan Shanmugasundram reviewed gene: ACTA2: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 WDR72 Achchuthan Shanmugasundram commented on gene: WDR72: This gene has been added to this panel with green rating as it was present in R257 Unexplained young onset end-stage renal disease panel (v5.1) with the same rating before it was made a super panel.
Unexplained young onset end-stage renal disease - additional genes v0.65 TRAP1 Achchuthan Shanmugasundram reviewed gene: TRAP1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 TBX18 Achchuthan Shanmugasundram reviewed gene: TBX18: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 SIX5 Achchuthan Shanmugasundram reviewed gene: SIX5: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 SALL1 Achchuthan Shanmugasundram reviewed gene: SALL1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 RRM2B Achchuthan Shanmugasundram reviewed gene: RRM2B: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 RMND1 Achchuthan Shanmugasundram commented on gene: RMND1: This gene has been added to this panel with green rating as it was present in R257 Unexplained young onset end-stage renal disease panel (v5.1) with the same rating before it was made a super panel.
Unexplained young onset end-stage renal disease - additional genes v0.65 RET Achchuthan Shanmugasundram edited their review of gene: RET: Added comment: This gene has been added to this panel with green rating as it was present in R257 Unexplained young onset end-stage renal disease panel (v5.1) with the same rating before it was made a super panel.; Changed rating: GREEN
Unexplained young onset end-stage renal disease - additional genes v0.65 PBX1 Achchuthan Shanmugasundram reviewed gene: PBX1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.65 LRIG2 Achchuthan Shanmugasundram reviewed gene: LRIG2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 KYNU Achchuthan Shanmugasundram reviewed gene: KYNU: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 ITGA8 Achchuthan Shanmugasundram reviewed gene: ITGA8: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 HPSE2 Achchuthan Shanmugasundram reviewed gene: HPSE2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 HAAO Achchuthan Shanmugasundram reviewed gene: HAAO: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 GRIP1 Achchuthan Shanmugasundram reviewed gene: GRIP1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 GLI3 Achchuthan Shanmugasundram reviewed gene: GLI3: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 GATA3 Achchuthan Shanmugasundram reviewed gene: GATA3: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 FREM2 Achchuthan Shanmugasundram reviewed gene: FREM2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 FREM1 Achchuthan Shanmugasundram reviewed gene: FREM1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 FRAS1 Achchuthan Shanmugasundram reviewed gene: FRAS1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 FAN1 Achchuthan Shanmugasundram reviewed gene: FAN1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 EYA1 Achchuthan Shanmugasundram reviewed gene: EYA1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 DSTYK Achchuthan Shanmugasundram reviewed gene: DSTYK: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 CHRM3 Achchuthan Shanmugasundram reviewed gene: CHRM3: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 CHD7 Achchuthan Shanmugasundram reviewed gene: CHD7: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 C3 Achchuthan Shanmugasundram reviewed gene: C3: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 BNC2 Achchuthan Shanmugasundram reviewed gene: BNC2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 ARMC9 Achchuthan Shanmugasundram reviewed gene: ARMC9: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 ANOS1 Achchuthan Shanmugasundram reviewed gene: ANOS1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: X-LINKED: hemizygous mutation in males, biallelic mutations in females
Unexplained young onset end-stage renal disease - additional genes v0.65 AGTR1 Achchuthan Shanmugasundram reviewed gene: AGTR1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 AGT Achchuthan Shanmugasundram reviewed gene: AGT: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.65 ACTG2 Achchuthan Shanmugasundram reviewed gene: ACTG2: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Unexplained young onset end-stage renal disease - additional genes v0.65 ACE Achchuthan Shanmugasundram reviewed gene: ACE: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Unexplained young onset end-stage renal disease - additional genes v0.64 TBX18 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.64 TBX18 Achchuthan Shanmugasundram gene: TBX18 was added
gene: TBX18 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: TBX18 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Phenotypes for gene: TBX18 were set to Congenital anomalies of kidney and urinary tract 2 143400
Unexplained young onset end-stage renal disease - additional genes v0.63 SIX5 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.63 SIX5 Achchuthan Shanmugasundram gene: SIX5 was added
gene: SIX5 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: SIX5 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: SIX5 were set to Branchiootorenal syndrome 2, 610896
Unexplained young onset end-stage renal disease - additional genes v0.62 SIX1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.62 SIX1 Achchuthan Shanmugasundram gene: SIX1 was added
gene: SIX1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: SIX1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Phenotypes for gene: SIX1 were set to Branchiootorenal Spectrum Disorders
Unexplained young onset end-stage renal disease - additional genes v0.61 SALL1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.61 SALL1 Achchuthan Shanmugasundram gene: SALL1 was added
gene: SALL1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: SALL1 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: SALL1 were set to imperforate anus, ear abnormalities, thumb abnormalities; Townes-Brocks branchiootorenal-like syndrome, 107480; Townes-Brocks syndrome, 107480
Unexplained young onset end-stage renal disease - additional genes v0.60 RRM2B Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.60 RRM2B Achchuthan Shanmugasundram gene: RRM2B was added
gene: RRM2B was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: RRM2B was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: RRM2B were set to Mitochondrial DNA depletion syndrome 8B (MNGIE type) 612075; Mitochondrial DNA depletion syndrome 8A (encephalomyopathic,renal tubulopathy), 612075; Mitochondrial DNA depletion syndrome 8A (encephalomyopathic type with renal tubulopathy) 612075
Unexplained young onset end-stage renal disease - additional genes v0.59 ROBO2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.59 ROBO2 Achchuthan Shanmugasundram gene: ROBO2 was added
gene: ROBO2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: ROBO2 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Phenotypes for gene: ROBO2 were set to Vesicoureteral Reflux; Vesicoureteral reflux 2, 610878
Unexplained young onset end-stage renal disease - additional genes v0.58 RET Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.58 RET Achchuthan Shanmugasundram gene: RET was added
gene: RET was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green,NHS GMS
Mode of inheritance for gene: RET was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: RET were set to Multiple endocrine neoplasia IIB, 162300; Central hypoventilation syndrome, congenital, 209880; Multiple endocrine neoplasia IIA, 171400; Renal agenesis, 191830; {Hirschsprung disease, susceptibility to, 1}, 142623; Pheochromocytoma, 171300; Renal Adysplasia; Medullary thyroid carcinoma, 155240
Unexplained young onset end-stage renal disease - additional genes v0.57 MYH11 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.57 MYH11 Achchuthan Shanmugasundram gene: MYH11 was added
gene: MYH11 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: MYH11 was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: MYH11 were set to Megacystis-microcolon-intestinal hypoperistalsis syndrome
Unexplained young onset end-stage renal disease - additional genes v0.56 LRIG2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.56 LRIG2 Achchuthan Shanmugasundram gene: LRIG2 was added
gene: LRIG2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: LRIG2 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: LRIG2 were set to Stuart HM, Roberts NA, Bergu B, Daly SB, Urquhart JE, Bhaskar S, Dickerson J, Mermerkaya M, Silay MS, Lewis MA, Olondriz BO, Gener B, Beetz C, Varga RE, G lp nar O, S er E, Yal nkaya F, G c k A, Yue WW, Erdogan F, Berry A, Hanley NA, McKenzie EA, Hilton EN, Woolf AS, Newman WG. LRIG2 mutations cause urofacial syndrome. Am J Hum Genet 92:259-264, 2013.
Phenotypes for gene: LRIG2 were set to Urofacial syndrome; Urofacial syndrome 2 615112; Congenital bladder disease: dyssynergic, high pressure bladder.
Unexplained young onset end-stage renal disease - additional genes v0.55 KYNU Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.55 KYNU Achchuthan Shanmugasundram gene: KYNU was added
gene: KYNU was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: KYNU was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: KYNU were set to 27604308; 17334708; 28792876
Phenotypes for gene: KYNU were set to ?Hydroxykynureninuria, 236800; multiple congenital malformations; VACTERL-like phenotype; Hydroxykynureninuria (Disorders of histidine, tryptophan or lysine metabolism)
Unexplained young onset end-stage renal disease - additional genes v0.54 ITGA8 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.54 ITGA8 Achchuthan Shanmugasundram gene: ITGA8 was added
gene: ITGA8 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: ITGA8 was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: ITGA8 were set to Renal hypodysplasia/aplasia 1, 191830
Unexplained young onset end-stage renal disease - additional genes v0.53 HPSE2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.53 HPSE2 Achchuthan Shanmugasundram gene: HPSE2 was added
gene: HPSE2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: HPSE2 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: HPSE2 were set to 20560210; 20560209
Phenotypes for gene: HPSE2 were set to Congenital bladder disease: dyssynergic, high pressure bladder; Urofacial syndrome 1 236730; Urofacial Syndrome
Unexplained young onset end-stage renal disease - additional genes v0.52 HAAO Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.52 HAAO Achchuthan Shanmugasundram gene: HAAO was added
gene: HAAO was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: HAAO was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: HAAO were set to 27604308; 17334708; 28792876
Phenotypes for gene: HAAO were set to VACTERL-like phenotype; Multiple congenital malformations
Unexplained young onset end-stage renal disease - additional genes v0.51 GRIP1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.51 GRIP1 Achchuthan Shanmugasundram gene: GRIP1 was added
gene: GRIP1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: GRIP1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: GRIP1 were set to 24700879; 14730302; 24357607; 22510445
Phenotypes for gene: GRIP1 were set to Fraser syndrome 219000; Fraser syndrome; isolated CAKUT
Unexplained young onset end-stage renal disease - additional genes v0.50 GLI3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.50 GLI3 Achchuthan Shanmugasundram gene: GLI3 was added
gene: GLI3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: GLI3 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: GLI3 were set to Pallister-Hall syndrome, OMIM:146510
Unexplained young onset end-stage renal disease - additional genes v0.49 FREM2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.49 FREM2 Achchuthan Shanmugasundram gene: FREM2 was added
gene: FREM2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: FREM2 was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: FREM2 were set to Fraser syndrome 219000; Fraser syndrome
Unexplained young onset end-stage renal disease - additional genes v0.48 FREM1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.48 FREM1 Achchuthan Shanmugasundram gene: FREM1 was added
gene: FREM1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: FREM1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: FREM1 were set to PMID: 24700879
Phenotypes for gene: FREM1 were set to Bifid nose with or without anorectal and renal anomalies, 608980
Unexplained young onset end-stage renal disease - additional genes v0.47 FRAS1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.47 FRAS1 Achchuthan Shanmugasundram gene: FRAS1 was added
gene: FRAS1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: FRAS1 was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: FRAS1 were set to Fraser syndrome 219000; Fraser syndrome
Unexplained young onset end-stage renal disease - additional genes v0.46 DSTYK Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.46 DSTYK Achchuthan Shanmugasundram gene: DSTYK was added
gene: DSTYK was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: DSTYK was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: DSTYK were set to Renal hypodysplasia; {Congenital anomalies of kidney and urinary tract, susceptibility to}; CONGENITAL ANOMALIES OF KIDNEY AND URINARY TRACT, CAKUT1; ureteropelvic junction obstruction; {Congenital anomalies of kidney and urinary tract, susceptibility to}, 610805; vesicoureteric reflux
Unexplained young onset end-stage renal disease - additional genes v0.45 CHD7 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.45 CHD7 Achchuthan Shanmugasundram gene: CHD7 was added
gene: CHD7 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: CHD7 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: CHD7 were set to CHARGE syndrome 214800
Unexplained young onset end-stage renal disease - additional genes v0.44 CHD1L Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.44 CHD1L Achchuthan Shanmugasundram gene: CHD1L was added
gene: CHD1L was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: CHD1L was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Publications for gene: CHD1L were set to 24429398; 22146311
Phenotypes for gene: CHD1L were set to ORPHA93545; Renal or urinary tract malformation (CAKUT)
Unexplained young onset end-stage renal disease - additional genes v0.43 ANOS1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.43 ANOS1 Achchuthan Shanmugasundram gene: ANOS1 was added
gene: ANOS1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: ANOS1 was set to X-LINKED: hemizygous mutation in males, biallelic mutations in females
Publications for gene: ANOS1 were set to 9719154; 11531922
Phenotypes for gene: ANOS1 were set to Hypogonadotropic hypogonadism 1 with or without anosmia (Kallmann syndrome 1); Kallman syndrome
Unexplained young onset end-stage renal disease - additional genes v0.42 AGTR1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.42 AGTR1 Achchuthan Shanmugasundram gene: AGTR1 was added
gene: AGTR1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: AGTR1 was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: AGTR1 were set to Renal Tubular Dysgenesis; Renal tubular dysgenesis, 267430; Hypertension, essential, 145500
Unexplained young onset end-stage renal disease - additional genes v0.41 AGT Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.41 AGT Achchuthan Shanmugasundram gene: AGT was added
gene: AGT was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: AGT was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: AGT were set to Renal Tubular Dysgenesis; {Hypertension, essential, susceptibility to}, 145500{Preeclampsia, susceptibility to}Renal tubular dysgenesis, 267430; Renal tubular dysgenesis, 267430
Unexplained young onset end-stage renal disease - additional genes v0.40 ACTG2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.40 ACTG2 Achchuthan Shanmugasundram gene: ACTG2 was added
gene: ACTG2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: ACTG2 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: ACTG2 were set to PMID: 25998219
Phenotypes for gene: ACTG2 were set to Berdon syndrome; visceral myopathy; Visceral myopathy (Megacystis-microcolon intestinal hypoperistalsis syndrome, Berdon syndrome) 155310; Megacystis-microcolon intestinal hypoperistalsis syndrome
Unexplained young onset end-stage renal disease - additional genes v0.39 ACTA2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.32
Unexplained young onset end-stage renal disease - additional genes v0.39 ACTA2 Achchuthan Shanmugasundram gene: ACTA2 was added
gene: ACTA2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: ACTA2 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: ACTA2 were set to Multi system smooth muscle dysfunction
Unexplained young onset end-stage renal disease - additional genes v0.38 WDR72 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.5
Unexplained young onset end-stage renal disease - additional genes v0.38 WDR72 Achchuthan Shanmugasundram gene: WDR72 was added
gene: WDR72 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green,NHS GMS,Expert Review
Mode of inheritance for gene: WDR72 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: WDR72 were set to 30028003; 30779877; 31959358; 33033857
Phenotypes for gene: WDR72 were set to hereditary distal renal tubular acidosis; distal renal tubular acidosis, MONDO:0015827; Amelogenesis imperfecta, type IIA3, OMIM:613211; amelogenesis imperfecta hypomaturation type 2A3, MONDO:0013181
Unexplained young onset end-stage renal disease - additional genes v0.37 UPK3A Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.5
Unexplained young onset end-stage renal disease - additional genes v0.37 UPK3A Achchuthan Shanmugasundram gene: UPK3A was added
gene: UPK3A was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: UPK3A was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Phenotypes for gene: UPK3A were set to Renal Adysplasia
Unexplained young onset end-stage renal disease - additional genes v0.36 UPK2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.5
Unexplained young onset end-stage renal disease - additional genes v0.36 UPK2 Achchuthan Shanmugasundram gene: UPK2 was added
gene: UPK2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: UPK2 was set to Other
Unexplained young onset end-stage renal disease - additional genes v0.35 TSHZ3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.35 TSHZ3 Achchuthan Shanmugasundram gene: TSHZ3 was added
gene: TSHZ3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: TSHZ3 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Unexplained young onset end-stage renal disease - additional genes v0.34 TRAP1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.34 TRAP1 Achchuthan Shanmugasundram gene: TRAP1 was added
gene: TRAP1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
gene-checked tags were added to gene: TRAP1.
Mode of inheritance for gene: TRAP1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: TRAP1 were set to 24152966
Phenotypes for gene: TRAP1 were set to CAKUT; VACTERL 192350
Unexplained young onset end-stage renal disease - additional genes v0.33 TNXB Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.33 TNXB Achchuthan Shanmugasundram gene: TNXB was added
gene: TNXB was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: TNXB was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.32 SPRY1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.32 SPRY1 Achchuthan Shanmugasundram gene: SPRY1 was added
gene: SPRY1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: SPRY1 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.31 SOX17 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.31 SOX17 Achchuthan Shanmugasundram gene: SOX17 was added
gene: SOX17 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: SOX17 was set to Unknown
Phenotypes for gene: SOX17 were set to Vesicoureteral reflux 3, 613674
Unexplained young onset end-stage renal disease - additional genes v0.30 SMARCA4 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.30 SMARCA4 Achchuthan Shanmugasundram gene: SMARCA4 was added
gene: SMARCA4 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: SMARCA4 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.29 SLIT2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.29 SLIT2 Achchuthan Shanmugasundram gene: SLIT2 was added
gene: SLIT2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: SLIT2 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.28 SHH Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.28 SHH Achchuthan Shanmugasundram gene: SHH was added
gene: SHH was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: SHH was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.27 RMND1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.27 RMND1 Achchuthan Shanmugasundram gene: RMND1 was added
gene: RMND1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green,NHS GMS,Literature
Mode of inheritance for gene: RMND1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: RMND1 were set to 31568715; 31889854; 32911714
Phenotypes for gene: RMND1 were set to Combined oxidative phosphorylation deficiency 11, OMIM:614922
Unexplained young onset end-stage renal disease - additional genes v0.26 PRDM15 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.26 PRDM15 Achchuthan Shanmugasundram gene: PRDM15 was added
gene: PRDM15 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Amber,Literature
Q2_24_promote_green tags were added to gene: PRDM15.
Mode of inheritance for gene: PRDM15 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: PRDM15 were set to 31950080; 33593823
Phenotypes for gene: PRDM15 were set to steroid-resistant nephrotic syndrome, MONDO:0044765
Unexplained young onset end-stage renal disease - additional genes v0.25 PBX1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.25 PBX1 Achchuthan Shanmugasundram gene: PBX1 was added
gene: PBX1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: PBX1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Publications for gene: PBX1 were set to 28270404; 28566479
Phenotypes for gene: PBX1 were set to CAKUT
Unexplained young onset end-stage renal disease - additional genes v0.24 NOS1AP Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.24 NOS1AP Achchuthan Shanmugasundram gene: NOS1AP was added
gene: NOS1AP was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Amber,Literature
Q1_24_promote_green tags were added to gene: NOS1AP.
Mode of inheritance for gene: NOS1AP was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: NOS1AP were set to 33523862
Phenotypes for gene: NOS1AP were set to Nephrotic syndrome, type 22, OMIM:619155
Unexplained young onset end-stage renal disease - additional genes v0.23 KIT Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.23 KIT Achchuthan Shanmugasundram gene: KIT was added
gene: KIT was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: KIT was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.22 ISCA-37401-Loss Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.22 ISCA-37401-Loss Achchuthan Shanmugasundram Region: ISCA-37401-Loss was added
Region: ISCA-37401-Loss was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for Region: ISCA-37401-Loss was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Phenotypes for Region: ISCA-37401-Loss were set to 194072; Wilms tumor, aniridia, genitourinary anomalies and mental retardation syndrome
Unexplained young onset end-stage renal disease - additional genes v0.21 HCN3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.21 HCN3 Achchuthan Shanmugasundram gene: HCN3 was added
gene: HCN3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: HCN3 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.20 GREM1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.20 GREM1 Achchuthan Shanmugasundram gene: GREM1 was added
gene: GREM1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: GREM1 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.19 GDNF Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.19 GDNF Achchuthan Shanmugasundram gene: GDNF was added
gene: GDNF was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: GDNF was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.18 GATA3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.18 GATA3 Achchuthan Shanmugasundram gene: GATA3 was added
gene: GATA3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: GATA3 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: GATA3 were set to Hypoparathyroidism, sensorineural deafness, and renal dysplasia, 146255; Hypoparathyroidism, Sensorineural Deafness, and Renal Disease
Unexplained young onset end-stage renal disease - additional genes v0.17 FOXC2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.17 FOXC2 Achchuthan Shanmugasundram gene: FOXC2 was added
gene: FOXC2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: FOXC2 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.16 FOXC1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.16 FOXC1 Achchuthan Shanmugasundram gene: FOXC1 was added
gene: FOXC1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: FOXC1 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.15 FAN1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.15 FAN1 Achchuthan Shanmugasundram gene: FAN1 was added
gene: FAN1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review,Expert Review Green
Mode of inheritance for gene: FAN1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: FAN1 were set to 22772369; 35931300
Phenotypes for gene: FAN1 were set to interstitial nephritis; chronic kidney disease; Interstitial nephritis, karyomegalic, OMIM:614817; karyomegalic interstitial nephritis, MONDO:0013898
Unexplained young onset end-stage renal disease - additional genes v0.14 EYA1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.14 EYA1 Achchuthan Shanmugasundram gene: EYA1 was added
gene: EYA1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: EYA1 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Phenotypes for gene: EYA1 were set to Branchiootorenal syndrome 1, with or without cataracts, 113650; Otofaciocervical syndrome, 166780; Branchiootic syndrome 1, 602588; Branchiootorenal syndrome 1, with or without cataracts; Branchiootorenal Spectrum Disorders; Anterior segment anomalies with or without cataract, 113650
Unexplained young onset end-stage renal disease - additional genes v0.13 DLG3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.13 DLG3 Achchuthan Shanmugasundram gene: DLG3 was added
gene: DLG3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: DLG3 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.12 DACT1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.12 DACT1 Achchuthan Shanmugasundram gene: DACT1 was added
gene: DACT1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: DACT1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Publications for gene: DACT1 were set to 28054444; 19701191; 22610794
Phenotypes for gene: DACT1 were set to ?Townes-Brocks syndrome 2,617466; TBS2
Unexplained young onset end-stage renal disease - additional genes v0.11 COX10 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.11 COX10 Achchuthan Shanmugasundram gene: COX10 was added
gene: COX10 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: COX10 was set to Unknown
Phenotypes for gene: COX10 were set to Mitochondrial complex IV deficiency, nuclear type 3, OMIM:619046; Encephalopathy, progressive mitochondrial, with proximal renal tubulopathy due tocytochrome c oxidase deficiency
Unexplained young onset end-stage renal disease - additional genes v0.10 CHRM3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.10 CHRM3 Achchuthan Shanmugasundram gene: CHRM3 was added
gene: CHRM3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: CHRM3 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: CHRM3 were set to 10944224; 22077972; 31441039
Phenotypes for gene: CHRM3 were set to Prune belly syndrome, OMIM:100100; Megacystis; Urinary Bladder Disease
Unexplained young onset end-stage renal disease - additional genes v0.9 C3 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.9 C3 Achchuthan Shanmugasundram gene: C3 was added
gene: C3 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: C3 was set to BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Publications for gene: C3 were set to 15781264; 18796626
Phenotypes for gene: C3 were set to C3 deficiency 613779 AR; {Hemolytic uremic syndrome, atypical, susceptibility to, 5} 612925 AD
Unexplained young onset end-stage renal disease - additional genes v0.8 BNC2 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.4
Unexplained young onset end-stage renal disease - additional genes v0.8 BNC2 Achchuthan Shanmugasundram gene: BNC2 was added
gene: BNC2 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green,Other
Mode of inheritance for gene: BNC2 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: BNC2 were set to 31051115
Phenotypes for gene: BNC2 were set to Posterior urethral valves; PUV; Congenital lower urinary-tract obstruction; Lower urinary tract obstruction, congenital, 618612
Mode of pathogenicity for gene: BNC2 was set to Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype - please provide details in the comments
Unexplained young onset end-stage renal disease - additional genes v0.7 BMP4 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.3
Unexplained young onset end-stage renal disease - additional genes v0.7 BMP4 Achchuthan Shanmugasundram gene: BMP4 was added
gene: BMP4 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: BMP4 was set to Unknown
Unexplained young onset end-stage renal disease - additional genes v0.6 BICC1 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.3
Unexplained young onset end-stage renal disease - additional genes v0.6 BICC1 Achchuthan Shanmugasundram gene: BICC1 was added
gene: BICC1 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Red
Mode of inheritance for gene: BICC1 was set to Unknown
Phenotypes for gene: BICC1 were set to {Renal dysplasia, cystic, susceptibility to}, 601331
Unexplained young onset end-stage renal disease - additional genes v0.5 ARMC9 Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.3
Unexplained young onset end-stage renal disease - additional genes v0.5 ARMC9 Achchuthan Shanmugasundram gene: ARMC9 was added
gene: ARMC9 was added to Unexplained young onset end-stage renal disease - additional genes. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: ARMC9 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: ARMC9 were set to 28625504
Phenotypes for gene: ARMC9 were set to Joubert syndrome 30, 617622
Unexplained young onset end-stage renal disease - additional genes v0.4 ACE Achchuthan Shanmugasundram Entity copied from Unexplained young onset end-stage renal disease v5.3
Unexplained young onset end-stage renal disease - additional genes v0.4 ACE Achchuthan Shanmugasundram gene: ACE was added
gene: ACE was added to Unexplained young onset end-stage renal disease - additional genes. Sources: Expert Review Green
Mode of inheritance for gene: ACE was set to BIALLELIC, autosomal or pseudoautosomal
Phenotypes for gene: ACE were set to Renal Tubular Dysgenesis; {Myocardial infarction, susceptibility to}{Alzheimer disease, susceptibility to}, 104300{Microvascular complications of diabetes 3}, 612624[Angiotensin I-converting enzyme, benign serum increase]{SARS, progression of}Renal tubular; Renal Tubular Dysgenesis 267430
Unexplained young onset end-stage renal disease - additional genes v0.3 Achchuthan Shanmugasundram Panel types changed to GMS Rare Disease Virtual; Component Of Super Panel
Unexplained young onset end-stage renal disease - additional genes v0.2 Achchuthan Shanmugasundram Panel types changed to GMS Rare Disease; Component Of Super Panel
Unexplained young onset end-stage renal disease - additional genes v0.1 Achchuthan Shanmugasundram Panel types changed to Component Of Super Panel
Unexplained young onset end-stage renal disease - additional genes v0.0 Achchuthan Shanmugasundram Added Panel Unexplained young onset end-stage renal disease - additional genes
Set panel types to: GMS Rare Disease Virtual; GMS Rare Disease; Component Of Super Panel