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Ataxia and cerebellar anomalies - narrow panel v5.3 THG1L Eleanor Williams Tag gene-checked tag was added to gene: THG1L.
Ataxia and cerebellar anomalies - narrow panel v5.3 THG1L Achchuthan Shanmugasundram Tag watchlist was removed from gene: THG1L.
Tag Q4_23_promote_green was removed from gene: THG1L.
Tag Q4_23_NHS_review was removed from gene: THG1L.
Ataxia and cerebellar anomalies - narrow panel v5.3 THG1L Sarah Leigh reviewed gene: THG1L: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Ataxia and cerebellar anomalies - narrow panel v5.2 THG1L Achchuthan Shanmugasundram Source Expert Review Green was added to THG1L.
Source NHS GMS was added to THG1L.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Ataxia and cerebellar anomalies - narrow panel v4.43 THG1L Achchuthan Shanmugasundram Classified gene: THG1L as Amber List (moderate evidence)
Ataxia and cerebellar anomalies - narrow panel v4.43 THG1L Achchuthan Shanmugasundram Added comment: Comment on list classification: There is sufficient evidence available for the promotion of this gene to green rating in the next GMS review.
Ataxia and cerebellar anomalies - narrow panel v4.43 THG1L Achchuthan Shanmugasundram Gene: thg1l has been classified as Amber List (Moderate Evidence).
Ataxia and cerebellar anomalies - narrow panel v4.42 THG1L Achchuthan Shanmugasundram Phenotypes for gene: THG1L were changed from Spinocerebellar ataxia, autosomal recessive 28, OMIM:618800; Spinocerebellar ataxia, autosomal recessive 28, MONDO:0032923 to Spinocerebellar ataxia, autosomal recessive 28, OMIM:618800, MONDO:0032923
Ataxia and cerebellar anomalies - narrow panel v4.41 THG1L Achchuthan Shanmugasundram Publications for gene: THG1L were set to 27307223; 30214071; 31168944
Ataxia and cerebellar anomalies - narrow panel v4.40 THG1L Achchuthan Shanmugasundram Tag Q4_23_promote_green tag was added to gene: THG1L.
Tag Q4_23_NHS_review tag was added to gene: THG1L.
Ataxia and cerebellar anomalies - narrow panel v4.40 THG1L Achchuthan Shanmugasundram reviewed gene: THG1L: Rating: GREEN; Mode of pathogenicity: None; Publications: 27307223, 30214071, 31168944, 33682303, 37670026; Phenotypes: Spinocerebellar ataxia, autosomal recessive 28, OMIM:618800; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Ataxia and cerebellar anomalies - narrow panel v4.40 THG1L Hannah Knight reviewed gene: THG1L: Rating: AMBER; Mode of pathogenicity: None; Publications: 33682303, 33682303; Phenotypes: Spinocerebellar ataxia, autosomal recessive 28; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Ataxia and cerebellar anomalies - narrow panel v2.51 THG1L Arina Puzriakova Tag watchlist tag was added to gene: THG1L.
Ataxia and cerebellar anomalies - narrow panel v2.51 THG1L Arina Puzriakova Phenotypes for gene: THG1L were changed from Cerebellar ataxia to Spinocerebellar ataxia, autosomal recessive 28, OMIM:618800; Spinocerebellar ataxia, autosomal recessive 28, MONDO:0032923
Ataxia and cerebellar anomalies - narrow panel v2.50 THG1L Arina Puzriakova Classified gene: THG1L as Amber List (moderate evidence)
Ataxia and cerebellar anomalies - narrow panel v2.50 THG1L Arina Puzriakova Added comment: Comment on list classification: Ataxia only reported in 3 Ashkenazi Jewish families with the same p.V55A founder variant. Unclear whether the fourth case with a different variant (p.L294P) displayed ataxia. Therefore, additional cases or functional analysis of the p.V55A variant are required prior to upgrading this gene to Green.
Ataxia and cerebellar anomalies - narrow panel v2.50 THG1L Arina Puzriakova Gene: thg1l has been classified as Amber List (Moderate Evidence).
Ataxia and cerebellar anomalies - narrow panel v2.49 THG1L Arina Puzriakova reviewed gene: THG1L: Rating: AMBER; Mode of pathogenicity: None; Publications: 27307223, 31168944, 30214071; Phenotypes: Spinocerebellar ataxia, autosomal recessive 28, OMIM:618800; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Ataxia and cerebellar anomalies - narrow panel v2.6 THG1L Zornitza Stark gene: THG1L was added
gene: THG1L was added to Ataxia and cerebellar anomalies - narrow panel. Sources: Literature
Mode of inheritance for gene: THG1L was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: THG1L were set to 27307223; 30214071; 31168944
Phenotypes for gene: THG1L were set to Cerebellar ataxia
Review for gene: THG1L was set to GREEN
Added comment: Four Ashkenazi Jewish families reported, with same homozygous variant, p.V55A in affected individuals. Another individual from different ethnicity also reported. A carrier rate of 0.8%, but no THG1L V55A homozygotes, was found in a cohort of 3,232 unrelated Ashkenazi Jewish individuals, and no homozygotes found in Exac or gnomAD.
Sources: Literature