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Intellectual disability v9.6 EPB41L3 Arina Puzriakova Classified gene: EPB41L3 as Amber List (moderate evidence)
Intellectual disability v9.6 EPB41L3 Arina Puzriakova Added comment: Comment on list classification: There is sufficient evidence to promote this gene to Green at the next GMS panel update.
Intellectual disability v9.6 EPB41L3 Arina Puzriakova Gene: epb41l3 has been classified as Amber List (Moderate Evidence).
Intellectual disability v9.5 EPB41L3 Arina Puzriakova Tag Q2_25_ promote_green tag was added to gene: EPB41L3.
Tag Q2_25_ NHS_review tag was added to gene: EPB41L3.
Intellectual disability v9.5 EPB41L3 Arina Puzriakova reviewed gene: EPB41L3: Rating: GREEN; Mode of pathogenicity: None; Publications: 39292993; Phenotypes: Developmental disorder with seizures and myelination defects; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Intellectual disability v9.5 EPB41L3 Arina Puzriakova Phenotypes for gene: EPB41L3 were changed from developmental delay; intellectual disability; seizures; hypotonia; neuroregression to Developmental disorder with seizures and myelination defects
Intellectual disability v8.243 EPB41L3 Julia Baptista gene: EPB41L3 was added
gene: EPB41L3 was added to Intellectual disability. Sources: Literature
Mode of inheritance for gene: EPB41L3 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: EPB41L3 were set to 39292993
Phenotypes for gene: EPB41L3 were set to developmental delay; intellectual disability; seizures; hypotonia; neuroregression
Review for gene: EPB41L3 was set to GREEN
Added comment: Six individuals from five unrelated families with global developmental delay, intellectual disability, seizures, hypotonia, neuroregression and delayed myelination. Exome sequencing identified biallelic variants in EPB41L3 in all affected individuals: two nonsense [c.466C>T, p.(R156*); c.2776C>T, p.(R926*)] and three frameshift [c.666delT, p.(F222Lfs*46); c.2289dupC, p.(V764Rfs*19); c.948_949delTG, p.(A317Kfs*33)].
Sources: Literature