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Severe early-onset obesity v5.18 STX16 Achchuthan Shanmugasundram Added comment: Comment on mode of inheritance: STX16 is not clearly an imprinted gene. As reported in publications, only maternally inherited deletions are associated with this phenotype. Deletions in this gene are thought to disrupt cis-acting regulation of GNAS expression.
Severe early-onset obesity v4.12 STX16 Dmitrijs Rots gene: STX16 was added
gene: STX16 was added to Severe early-onset obesity. Sources: Literature
Mode of inheritance for gene: STX16 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: STX16 were set to PMID: 27338644; 28453643
Phenotypes for gene: STX16 were set to Obesity with pseudohypoparathyroidism type 1B
Penetrance for gene: STX16 were set to Incomplete
Review for gene: STX16 was set to GREEN
Added comment: PMID: 27338644 & PMID: 28453643 shows that STX16 deletions, causing GNAS methylation defects, result in obesity. GNAS is already on this panel as green. Enough evidence for the green rating.
Sources: Literature
Severe early-onset obesity v4.12 GNAS Achchuthan Shanmugasundram Tag Q4_22_MOI was removed from gene: GNAS.
Severe early-onset obesity v4.12 GNAS Achchuthan Shanmugasundram reviewed gene: GNAS: Rating: ; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Severe early-onset obesity v4.11 GNAS Achchuthan Shanmugasundram Source NHS GMS was added to GNAS.
Mode of inheritance for gene GNAS was changed from MONOALLELIC, autosomal or pseudoautosomal, paternally imprinted (maternal allele expressed) to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Severe early-onset obesity v2.52 GNAS Sarah Leigh reviewed gene: GNAS: Rating: ; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Severe early-onset obesity v2.52 GNAS Sarah Leigh Phenotypes for gene: GNAS were changed from Congenital Obesity; Pseudohypoparathyroidism Ia, OMIM:103580; Pseudohypoparathyroidism Ib, OMIM:603233; Pseudohypoparathyroidism Ic, OMIM:612462 to Pseudohypoparathyroidism Ia, OMIM:103580; pseudohypoparathyroidism type 1A, MONDO:0007078; Pseudohypoparathyroidism Ic, OMIM:612462; pseudohypoparathyroidism type 1C, MONDO:0012911; Pseudopseudohypoparathyroidism, OMIM:612463; pseudopseudohypoparathyroidism, MONDO:0012912
Severe early-onset obesity v2.51 GNAS Sarah Leigh Tag Q4_22_MOI tag was added to gene: GNAS.
Severe early-onset obesity v2.15 GNAS Ivone Leong Phenotypes for gene: GNAS were changed from Congenital Obesity; Pseudohypoparathyroidism Ia, 103580; Pseudohypoparathyroidism Ib, 603233; Pseudohypoparathyroidism Ic, 612462 to Congenital Obesity; Pseudohypoparathyroidism Ia, OMIM:103580; Pseudohypoparathyroidism Ib, OMIM:603233; Pseudohypoparathyroidism Ic, OMIM:612462
Severe early-onset obesity v1.22 GNAS Ivone Leong reviewed gene: GNAS: Rating: GREEN; Mode of pathogenicity: ; Publications: 28453643, 27991864, 28663568; Phenotypes: ; Mode of inheritance:
Severe early-onset obesity v1.22 GNAS Ismaa Farooqi reviewed gene: GNAS: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, paternally imprinted (maternal allele expressed); Current diagnostic: yes
Severe early-onset obesity v1.21 GNAS Ivone Leong Source Expert Review Green was added to GNAS.
Source Expert list was added to GNAS.
Mode of inheritance for gene GNAS was changed from to MONOALLELIC, autosomal or pseudoautosomal, paternally imprinted (maternal allele expressed)
Publications for gene GNAS were changed from to 27991864; 28663568; 28453643
Rating Changed from Red List (low evidence) to Green List (high evidence)
Severe early-onset obesity v1.15 GNAS Ivone Leong Phenotypes for gene: GNAS were changed from Congenital Obesity; Pseudohypoparathyroidism Ib, 603233; Pseudohypoparathyroidism Ic, 612462 to Congenital Obesity; Pseudohypoparathyroidism Ia, 103580; Pseudohypoparathyroidism Ib, 603233; Pseudohypoparathyroidism Ic, 612462
Severe early-onset obesity v1.14 GNAS Ivone Leong Phenotypes for gene: GNAS were changed from Congenital Obesity; Pseudopseudohypoparathyroidism, 612463 to Congenital Obesity; Pseudohypoparathyroidism Ib, 603233; Pseudohypoparathyroidism Ic, 612462
Severe early-onset obesity v1.13 GNAS Ivone Leong Phenotypes for gene: GNAS were changed from Congenital Obesity; Pseudohypoparathyroidism, 612463 to Congenital Obesity; Pseudopseudohypoparathyroidism, 612463
Severe early-onset obesity v1.12 GNAS Ivone Leong Phenotypes for gene: GNAS were changed from Congenital Obesity; Pseudopseudohypoparathyroidism, 612463 to Congenital Obesity; Pseudohypoparathyroidism, 612463
Severe early-onset obesity v1.11 GNAS Ivone Leong Phenotypes for gene: GNAS were changed from Congenital Obesity to Congenital Obesity; Pseudopseudohypoparathyroidism, 612463