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Severe microcephaly v7.12 FRA10AC1 Achchuthan Shanmugasundram Tag Q3_24_promote_green was removed from gene: FRA10AC1.
Severe microcephaly v7.12 FRA10AC1 Achchuthan Shanmugasundram commented on gene: FRA10AC1: The rating of this gene has been updated to green following NHS Genomic Medicine Service approval.
Severe microcephaly v7.11 FRA10AC1 Achchuthan Shanmugasundram Source NHS GMS was added to FRA10AC1.
Source Expert Review Green was added to FRA10AC1.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Severe microcephaly v5.17 FRA10AC1 Achchuthan Shanmugasundram Classified gene: FRA10AC1 as Amber List (moderate evidence)
Severe microcephaly v5.17 FRA10AC1 Achchuthan Shanmugasundram Added comment: Comment on list classification: There is sufficient evidence available (three unrelated families) for the promotion of this gene to green rating in the next GMS update.
Severe microcephaly v5.17 FRA10AC1 Achchuthan Shanmugasundram Gene: fra10ac1 has been classified as Amber List (Moderate Evidence).
Severe microcephaly v5.16 FRA10AC1 Achchuthan Shanmugasundram Tag Q3_24_promote_green tag was added to gene: FRA10AC1.
Severe microcephaly v5.16 FRA10AC1 Achchuthan Shanmugasundram gene: FRA10AC1 was added
gene: FRA10AC1 was added to Severe microcephaly. Sources: Literature
Mode of inheritance for gene: FRA10AC1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: FRA10AC1 were set to 34694367
Phenotypes for gene: FRA10AC1 were set to Neurodevelopmental disorder with growth retardation, dysmorphic facies, and corpus callosum abnormalities, OMIM:620113
Review for gene: FRA10AC1 was set to GREEN
Added comment: PMID:34694367 reported the identification of homozygous FRA10AC1 variants in five individuals from three unrelated consanguineous Arabic families with a neurodevelopmental disorder. The two unrelated patients from two different families with loss-of-function variants (g.4656_7575del and c.561_562insTTTA/ p.Ser188Phefs*6) presented with developmental delay, profound intellectual disability (ID), and no speech, while three siblings from the third family with the c.494_496delAAG (p.Glu165del) variant had borderline to mild ID. All five individuals had microcephaly and the Z-score of OFC was <-3 for four individuals from three families.

This gene has been associated with relevant phenotypes in OMIM (MIM #620113) and Gene2Phenotype ('strong' rating on the DD panel).
Sources: Literature