- AARS2 3
- AASS 3
- ABAT 5
- ABCA1 1
- ABCB11 1
- ABCB4 1
- ABCB7 5
- ABCD1 2
- ABCD4 1
- ABCG5 1
- ABCG8 1
- ABHD12 1
- ABHD5 1
- ACACA 2
- ACAD8 1
- ACAD9 4
- ACADM 3
- ACADS 1
- ACADSB 1
- ACADVL 3
- ACAT1 1
- ACO2 5
- ACOX1 0
- ACOX2 2
- ACSF3 3
- ACY1 1
- ADA 3
- ADAR 1
- ADSL 1
- AFG3L2 4
- AGA 1
- AGK 3
- AGL 1
- AGPS 0
- AGXT 0
- AHCY 2
- AIFM1 2
- AKR1D1 1
- ALAD 1
- ALAS2 1
- ALDH18A1 2
- ALDH3A2 1
- ALDH4A1 1
- ALDH5A1 1
- ALDH6A1 1
- ALDH7A1 2
- ALDOA 1
- ALDOB 0
- ALG1 0
- ALG11 0
- ALG12 0
- ALG14 1
- ALG3 0
- ALG6 0
- ALG8 0
- ALG9 0
- ALPL 1
- AMACR 0
- AMN 1
- AMT 1
- ANO10 3
- APOA1 1
- APOA5 2
- APOB 2
- APOC2 1
- APOE 1
- APOPT1 3
- APRT 1
- APTX 3
- ARG1 0
- ARSA 1
- ARSB 0
- ARSE 1
- ARSG 3
- ARSK 2
- ASAH1 1
- ASL 0
- ASPA 1
- ASS1 0
- ATAD3A 5
- ATIC 2
- ATP13A2 1
- ATP5A1 6
- ATP5D 1
- ATP5E 4
- ATP5G3 5
- ATP5O 4
- ATP6AP1 0
- ATP6V0A2 0
- ATP7A 3
- ATP7B 1
- ATP8B1 1
- ATPAF2 2
- AUH 0
- B3GALNT2 0
- B3GALT6 0
- B3GAT3 0
- B3GLCT 1
- B4GALNT1 2
- B4GALT1 0
- B4GALT7 0
- BAAT 1
- BCAT2 3
- BCKDHA 0
- BCKDHB 0
- BCKDK 1
- BCS1L 2
- BOLA3 3
- BTD 2
- C12orf65 3
- C19orf12 7
- C19orf70 2
- C1QBP 2
- C2orf69 4
- CA5A 2
- CARS2 4
- CAT 0
- CBS 2
- CCDC115 1
- CHCHD10 3
- CHKB 6
- CHST14 0
- CHST3 0
- CHST6 0
- CHSY1 0
- CISD2 1
- CLDN16 1
- CLDN19 1
- CLN3 1
- CLN5 1
- CLN6 1
- CLN8 1
- CLPB 5
- CLPP 2
- CMPK2 3
- CNNM2 1
- COA6 3
- COA7 1
- COG1 0
- COG4 0
- COG5 0
- COG6 0
- COG7 0
- COG8 0
- COQ2 3
- COQ4 2
- COQ6 2
- COQ7 3
- COQ8A 4
- COQ8B 3
- COQ9 2
- COX10 2
- COX11 4
- COX14 3
- COX15 2
- COX18 4
- COX20 3
- COX4I1 3
- COX5A 3
- COX6A1 2
- COX6A2 4
- COX6B1 1
- COX7B 2
- CP 1
- CPOX 4
- CPS1 0
- CPT1A 1
- CPT2 4
- CREB3L3 4
- CRLS1 2
- CSGALNACT1 3
- CTH 1
- CTNS 1
- CTSA 1
- CTSC 1
- CTSD 1
- CTSF 1
- CTSK 1
- CUBN 1
- CYC1 2
- CYCS 5
- CYP27A1 1
- CYP7B1 1
- D2HGDH 1
- DARS 7
- DARS2 3
- DBH 2
- DBT 0
- DCXR 2
- DDC 2
- DDOST 2
- DGUOK 2
- DHCR24 1
- DHCR7 2
- DHFR 0
- DHODH 1
- DHRSX 7
- DHTKD1 7
- DLAT 3
- DLD 3
- DNA2 3
- DNAJC12 1
- DNAJC19 3
- DNAJC5 1
- DNM1L 3
- DNM2 3
- DOLK 0
- DPAGT1 0
- DPM1 0
- DPM2 2
- DPM3 2
- DPYD 2
- DPYS 1
- DYM 2
- EARS2 2
- EBP 1
- ECHS1 3
- EDEM3 1
- EHBP1L1 2
- ELAC2 2
- ENO3 2
- EOGT 2
- EPG5 1
- EPM2A 1
- ETFA 2
- ETFB 2
- ETFDH 3
- ETHE1 2
- EXT1 1
- EXT2 0
- FA2H 1
- FAH 1
- FARS2 3
- FASTKD2 2
- FBP1 0
- FBXL4 4
- FDX2 6
- FDXR 2
- FECH 3
- FGFR2 1
- FH 2
- FKRP 0
- FKTN 0
- FLAD1 3
- FMO3 1
- FOLR1 0
- FOXRED1 1
- FTCD 2
- FUCA1 1
- FUK 3
- FUT8 1
- FXN 7
- G6PC 2
- G6PC3 1
- GAA 2
- GABRG2 1
- GALC 1
- GALE 1
- GALK1 1
- GALM 2
- GALNS 0
- GALNT2 3
- GALNT3 0
- GALT 1
- GAMT 2
- GARS 3
- GATM 3
- GBA 1
- GBE1 1
- GCDH 1
- GCH1 1
- GCLC 1
- GCSH 4
- GDAP1 4
- GFER 2
- GFM1 2
- GFM2 3
- GFPT1 0
- GIF 2
- GK 1
- GLA 0
- GLB1 0
- GLDC 1
- GLRA1 1
- GLRX5 3
- GLS 4
- GLUD1 5
- GLUL 1
- GLYCTK 1
- GM2A 1
- GMPPA 2
- GMPPB 0
- GNE 2
- GNMT 1
- GNPAT 0
- GNPTAB 0
- GNPTG 0
- GNS 0
- GORAB 2
- GPD1 1
- GPHN 1
- GPIHBP1 1
- GRHPR 0
- GRN 3
- GSS 1
- GSTZ1 2
- GTPBP3 3
- GUK1 3
- GUSB 0
- GYG1 2
- GYS1 2
- GYS2 0
- HAAO 1
- HADH 1
- HADHA 2
- HADHB 2
- HAMP 1
- HARS2 5
- HCCS 2
- HCFC1 1
- HEXA 1
- HEXB 1
- HFE 1
- HFE2 2
- HGD 1
- HGSNAT 0
- HIBCH 3
- HLCS 2
- HMBS 4
- HMGCL 4
- HMGCS2 1
- HOGA1 0
- HPD 1
- HPDL 3
- HPRT1 1
- HPS1 1
- HS2ST1 2
- HSD11B2 1
- HSD17B10 2
- HSD17B4 0
- HSD3B7 2
- HSPA9 5
- HSPD1 2
- HTRA2 1
- HYAL1 2
- IARS2 3
- IBA57 2
- IDH1 3
- IDH2 2
- IDH3A 3
- IDS 0
- IDUA 0
- IER3IP1 6
- ISCA1 1
- ISCA2 5
- ISCU 4
- ISPD 1
- ITPA 1
- IVD 0
- KARS 5
- KIAA0391 3
- KYNU 2
- L2HGDH 1
- LAMP2 2
- LARGE1 2
- LARS 3
- LARS2 2
- LBR 1
- LCAT 1
- LCT 1
- LDHA 1
- LDLR 1
- LDLRAP1 1
- LETM1 4
- LFNG 3
- LIAS 3
- LIG3 4
- LIPA 1
- LIPT1 3
- LIPT2 4
- LMBRD1 1
- LMF1 1
- LONP1 2
- LPIN1 2
- LPL 1
- LRPPRC 3
- LYRM4 4
- LYRM7 3
- MAGT1 1
- MAN1B1 0
- MAN2B1 1
- MAN2B2 3
- MANBA 1
- MAOA 1
- MARS2 2
- MAT1A 2
- MCCC1 1
- MCCC2 1
- MCEE 0
- MCOLN1 0
- MDH2 1
- MECR 2
- MFF 2
- MFN2 3
- MFSD8 1
- MGAT2 0
- MGME1 3
- MICU1 2
- MIPEP 1
- MLYCD 0
- MMAA 0
- MMAB 0
- MMACHC 1
- MMADHC 1
- MOCS1 1
- MOCS2 1
- MOGS 1
- MPC1 3
- MPDU1 0
- MPI 0
- MPV17 2
- MRM2 2
- MRPL3 5
- MRPL39 3
- MRPL44 3
- MRPL49 2
- MRPS2 2
- MRPS22 2
- MRPS34 2
- MSMO1 1
- MSTO1 3
- MT-ATP6 3
- MT-ATP8 3
- MT-CO1 3
- MT-CO2 3
- MT-CO3 3
- MT-CYB 3
- MTFMT 4
- MTHFR 0
- MT-ND1 3
- MT-ND2 3
- MT-ND3 3
- MT-ND4 3
- MT-ND4L 3
- MT-ND5 3
- MT-ND6 3
- MTO1 2
- MTPAP 5
- MTR 1
- MT-RNR1 3
- MTRR 1
- MT-TA 3
- MT-TC 3
- MT-TD 3
- MT-TE 3
- MT-TF 3
- MT-TG 3
- MT-TH 3
- MT-TI 3
- MT-TK 3
- MT-TL1 3
- MT-TL2 3
- MT-TM 3
- MT-TN 3
- MTTP 1
- MT-TP 3
- MT-TQ 3
- MT-TR 3
- MT-TS1 3
- MT-TS2 3
- MT-TT 5
- MT-TV 3
- MT-TW 3
- MT-TY 3
- MUT 1
- MVK 1
- NADK2 3
- NAGA 1
- NAGLU 0
- NAGS 0
- NARS2 2
- NAXD 3
- NAXE 2
- NDUFA1 2
- NDUFA10 1
- NDUFA11 1
- NDUFA12 4
- NDUFA13 6
- NDUFA2 1
- NDUFA4 4
- NDUFA6 3
- NDUFA8 5
- NDUFA9 5
- NDUFAF1 1
- NDUFAF2 2
- NDUFAF3 1
- NDUFAF4 1
- NDUFAF5 2
- NDUFAF6 3
- NDUFAF8 1
- NDUFB10 4
- NDUFB11 2
- NDUFB3 2
- NDUFB7 4
- NDUFB8 2
- NDUFC2 2
- NDUFS1 1
- NDUFS2 1
- NDUFS3 1
- NDUFS4 1
- NDUFS6 1
- NDUFS7 1
- NDUFS8 1
- NDUFV1 1
- NDUFV2 2
- NEU1 0
- NFS1 6
- NFU1 3
- NGLY1 2
- NHLRC1 1
- NNT 2
- NPC1 1
- NPC2 1
- NSDHL 1
- NSUN3 3
- NT5C3A 1
- NT5E 2
- NUBPL 2
- NUS1 4
- OAT 0
- OCRL 2
- OGDH 5
- OPA1 4
- OPA3 2
- OTC 1
- OXCT1 1
- PAH 1
- PANK2 2
- PARS2 4
- PC 3
- PCBD1 1
- PCCA 0
- PCCB 0
- PCK1 2
- PCSK9 1
- PDE12 3
- PDHA1 3
- PDHB 3
- PDHX 3
- PDK3 6
- PDP1 3
- PDSS1 2
- PDSS2 2
- PEPD 1
- PET100 4
- PEX1 0
- PEX10 0
- PEX11B 0
- PEX12 0
- PEX13 0
- PEX14 1
- PEX16 0
- PEX19 0
- PEX2 0
- PEX26 0
- PEX3 0
- PEX5 1
- PEX6 2
- PEX7 0
- PFKM 2
- PGAM2 2
- PGAP2 0
- PGAP3 0
- PGK1 2
- PGM1 1
- PGM3 0
- PHGDH 1
- PHKA1 2
- PHKA2 0
- PHKB 1
- PHKG2 0
- PHYH 0
- PIGA 0
- PIGL 0
- PIGM 4
- PIGN 0
- PIGO 0
- PIGS 2
- PIGT 1
- PIGV 0
- PIGW 1
- PINK1 1
- PITRM1 5
- PLA2G6 1
- PMM2 0
- PMPCA 2
- PMPCB 1
- PNP 1
- PNPLA2 3
- PNPLA8 3
- PNPO 1
- PNPT1 2
- POLG 4
- POLG2 4
- POLRMT 4
- POMGNT1 0
- POMGNT2 0
- POMK 2
- POMT1 0
- POMT2 0
- POR 1
- PPA2 1
- PPOX 3
- PPT1 1
- PRKAG2 2
- PRODH 2
- PRPS1 1
- PSAP 0
- PSAT1 1
- PTCD3 2
- PTS 2
- PUS1 4
- PYCR1 3
- PYGL 0
- PYGM 1
- QARS 5
- QDPR 1
- QRSL1 3
- RARS2 3
- RBCK1 2
- RBP4 2
- RFT1 0
- RMND1 3
- RNASEH1 3
- RNASEH2A 4
- RNASEH2B 4
- RNASEH2C 4
- RPIA 2
- RPL10 1
- RRM2B 3
- RTN4IP1 2
- SACS 2
- SAMHD1 3
- SAR1B 2
- SARS2 2
- SC5D 2
- SCO1 2
- SCO2 2
- SCP2 0
- SDHA 4
- SDHAF1 2
- SDHB 3
- SDHD 3
- SEC23B 2
- SERAC1 3
- SETX 2
- SFXN4 3
- SGSH 0
- SHMT2 2
- SI 2
- SKIV2L 2
- SLC12A3 2
- SLC13A3 3
- SLC16A1 1
- SLC17A5 1
- SLC18A2 2
- SLC19A2 3
- SLC19A3 3
- SLC22A5 2
- SLC25A1 4
- SLC25A12 4
- SLC25A13 1
- SLC25A15 0
- SLC25A19 3
- SLC25A20 1
- SLC25A22 3
- SLC25A24 3
- SLC25A26 2
- SLC25A3 3
- SLC25A32 1
- SLC25A36 3
- SLC25A38 2
- SLC25A4 3
- SLC25A42 3
- SLC25A46 2
- SLC2A1 2
- SLC2A2 0
- SLC30A10 1
- SLC35A1 2
- SLC35A2 3
- SLC35C1 0
- SLC35D1 0
- SLC37A4 2
- SLC39A14 1
- SLC39A4 2
- SLC39A8 2
- SLC3A1 2
- SLC40A1 1
- SLC46A1 0
- SLC52A2 2
- SLC52A3 1
- SLC5A1 2
- SLC5A6 2
- SLC6A19 3
- SLC6A3 2
- SLC6A8 2
- SLC7A7 0
- SLC7A9 2
- SMPD1 1
- SPATA5 5
- SPG7 7
- SPR 2
- SPTLC1 3
- SPTLC2 2
- SQOR 3
- SRD5A3 0
- SSBP1 3
- SSR4 0
- ST3GAL3 2
- ST3GAL5 0
- STS 2
- STT3A 3
- SUCLA2 3
- SUCLG1 2
- SUMF1 1
- SUOX 1
- SUPV3L1 2
- SURF1 3
- TACO1 2
- TALDO1 1
- TAMM41 3
- TANGO2 5
- TARS2 3
- TAT 2
- TAZ 3
- TCN2 2
- TEFM 3
- TFAM 3
- TFR2 1
- TH 2
- TIMM50 2
- TIMM8A 2
- TIMMDC1 3
- TK2 3
- TMEM126B 4
- TMEM165 0
- TMEM199 2
- TMEM5 1
- TMEM70 1
- TOMM7 3
- TOP3A 2
- TPK1 3
- TPP1 1
- TRAP1 2
- TREX1 2
- TRIM37 0
- TRIT1 3
- TRMT10C 3
- TRMT5 4
- TRMU 3
- TRNT1 2
- TRPM6 1
- TRPM7 2
- TSFM 3
- TTC19 1
- TTC37 3
- TTPA 2
- TUFM 6
- TUSC3 0
- TWNK 5
- TYMP 3
- UGGT1 2
- UGT1A1 3
- UMOD 2
- UMPS 1
- UPB1 3
- UQCC2 5
- UQCRB 5
- UQCRC2 6
- UQCRFS1 3
- UROD 1
- UROS 2
- VARS2 2
- VIPAS39 2
- VKORC1 2
- VPS16 1
- VPS33A 1
- VPS33B 2
- WARS2 3
- WDR45 1
- WFS1 3
- XDH 1
- XPNPEP3 4
- XYLT1 1
- XYLT2 0
- YARS2 2
- ALG13 1
- ALG2 2
- ATP5B 4
- CD320 2
- CLCN7 1
- COA3 3
- COASY 1
- COG3 2
- COX4I2 3
- CSTB 1
- DCC 1
- DHDDS 1
- EHHADH 2
- ERAL1 1
- GATC 2
- LDHD 2
- LIPC 2
- MRPS14 1
- MRPS16 5
- MRPS7 2
- MT-RNR2 3
- NDUFB9 4
- OPLAH 2
- PAICS 1
- PET117 1
- PSPH 2
- RANBP2 2
- RNASET2 2
- RYR1 1
- SDHAF2 4
- SDHC 3
- SLC31A1 1
- SSR3 2
- STAT2 7
- TKFC 1
- TMEM65 1
- TRAPPC11 1
- UQCC3 3
- UQCRC1 2
- UQCRQ 2
- UROC1 2
- YME1L1 1
- ABCG2 1
- ACAT2 2
- ALDH1B1 2
- ALG10 2
- AMPD1 2
- AOX1 1
- ATAD3B 2
- ATP5C1 3
- ATP5G1 3
- ATP5G2 3
- ATP5I 3
- ATP5J 3
- ATXN7 2
- BCAT1 1
- BOLA1 1
- BOLA2 1
- C1GALT1C1 1
- CAD 0
- CAMLG 1
- CEP89 3
- CETP 1
- CLCN6 1
- CLPS 1
- CNDP1 1
- COA1 2
- COA5 4
- COG2 0
- COX5B 1
- COX6C 1
- COX7A1 1
- COX7A2 1
- COX7B2 1
- COX7C 1
- COX8A 3
- CYP7A1 1
- DHFR2 1
- DLST 3
- DMGDH 1
- DPEP1 1
- DTD1 0
- ECSIT 2
- EGF 1
- ERCC6L2 0
- FAR1 2
- FBP2 1
- FOLR2 1
- FOLR3 1
- FXYD2 1
- GALNT12 1
- GATB 2
- GGT1 2
- HAL 1
- HYKK 1
- IDH3B 2
- KCTD7 1
- KHK 1
- LACTB 1
- LIPI 1
- MRPL12 4
- MRPL40 2
- MRPS23 4
- MTHFD1 1
- NAT8L 1
- NDUFA3 2
- NDUFA5 1
- NDUFA7 1
- NDUFAB1 1
- NDUFB1 1
- NDUFB2 1
- NDUFB4 1
- NDUFB5 1
- NDUFB6 1
- NDUFC1 1
- NDUFS5 2
- NDUFV3 2
- NT5C 1
- NUP62 1
- OSTC 2
- OXA1L 2
- PCYT2 1
- PDK1 3
- PDK2 2
- PDK4 2
- PDP2 4
- PDPR 5
- PDXK 1
- PEX11A 1
- PHKG1 1
- PHYKPL 1
- PNLIP 1
- PNPLA4 1
- POP1 1
- PPM1B 1
- PPM1K 1
- PREPL 2
- PTCD1 1
- PTPRZ1 1
- ROBO3 4
- SARDH 1
- SCARB1 1
- SHPK 1
- SLC22A4 1
- SLC25A2 1
- SLC25A40 1
- SLC26A6 1
- SLC27A5 1
- SLC35A3 0
- SLC36A2 2
- SLC52A1 1
- SLC6A20 2
- SLCO1B1 1
- SLCO1B3 1
- SRRT 2
- STT3B 0
- SUCLG2 2
- SUGCT 1
- TCN1 2
- TDO2 1
- TIMM44 1
- TM6SF2 1
- TMEM126A 3
- TPMT 1
- TREH 1
- TXN2 4
- UQCRH 1
- USF1 1
- VPS13C 5
Likely inborn error of metabolism
Gene: FKRP Green List (high evidence)EnsemblGeneIds (GRCh38): ENSG00000181027
EnsemblGeneIds (GRCh37): ENSG00000181027
OMIM: 606596, Gene2Phenotype
FKRP is in 21 panels
0 reviews
Details
- Mode of Inheritance
- BIALLELIC, autosomal or pseudoautosomal
- Sources
-
- London North GLH
- NHS GMS
- Expert Review Green
- Phenotypes
-
- Muscular dystrophy-dystroglycanopathy (congenital with or without mental retardation), type B, 5 606612
- Fukutin-related protein deficiency (Disorders of protein O-glycosylation, O-mannosylglycan synthesis deficiencies)
- Muscular dystrophy-dystroglycanopathy (limb-girdle), type C, 5 607155
- Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 5 613153
- OMIM
- 606596
- Clinvar variants
- Variants in FKRP
- Penetrance
- None
- Publications
- Panels with this gene
-
- Undiagnosed metabolic disorders
- Congenital disorders of glycosylation
- Early onset or syndromic epilepsy
- Cerebellar hypoplasia
- Structural eye disease
- Dilated and arrhythmogenic cardiomyopathy
- Paediatric or syndromic cardiomyopathy
- Malformations of cortical development
- Hydrocephalus
- Ataxia and cerebellar anomalies - narrow panel
- Likely inborn error of metabolism
- Intellectual disability
- Rhabdomyolysis and metabolic muscle disorders
- Arthrogryposis
- Childhood onset dystonia, chorea or related movement disorder
- DDG2P
- Acute rhabdomyolysis
- Congenital muscular dystrophy
- Limb girdle muscular dystrophies, myofibrillar myopathies and distal myopathies
- Fetal anomalies
- Bilateral congenital or childhood onset cataracts
History Filter Activity
Added New Source, Added New Source
Ivone Leong (Genomics England Curator)Source NHS GMS was added to FKRP. Source London North GLH was added to FKRP.
Panel promoted to version 1.0
Ellen McDonagh (Genomics England Curator)Checked against super panel made up of the panel constituents. Ready to promote to version 1.
Set Phenotypes, Set publications
Ellen McDonagh (Genomics England Curator)Added phenotypes Muscular dystrophy-dystroglycanopathy (congenital with or without mental retardation), type B, 5 606612; Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 5 613153; Muscular dystrophy-dystroglycanopathy (limb-girdle), type C, 5 607155; Fukutin-related protein deficiency (Disorders of protein O-glycosylation, O-mannosylglycan synthesis deficiencies) for gene: FKRP Publications for gene FKRP were changed from 27421908 to 27604308
Created, Added New Source, Set mode of inheritance, Set publications, Set Phenotypes
Ellen McDonagh (Genomics England Curator)gene: FKRP was added gene: FKRP was added to Inborn errors of metabolism. Sources: Expert Review Green Mode of inheritance for gene: FKRP was set to BIALLELIC, autosomal or pseudoautosomal Publications for gene: FKRP were set to 27421908 Phenotypes for gene: FKRP were set to Muscular dystrophy-dystroglycanopathy (congenital with or without mental retardation), type B, 5 606612; Muscular dystrophy-dystroglycanopathy (limb-girdle), type C, 5 607155; Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 5 613153