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Mendeliome v2.95 SF3B3 Zornitza Stark Marked gene: SF3B3 as ready
Mendeliome v2.95 SF3B3 Zornitza Stark Gene: sf3b3 has been classified as Green List (High Evidence).
Mendeliome v2.95 SF3B3 Zornitza Stark Classified gene: SF3B3 as Green List (high evidence)
Mendeliome v2.95 SF3B3 Zornitza Stark Gene: sf3b3 has been classified as Green List (High Evidence).
Mendeliome v2.94 SF3B3 Zornitza Stark gene: SF3B3 was added
gene: SF3B3 was added to Mendeliome. Sources: Literature
Mode of inheritance for gene: SF3B3 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: SF3B3 were set to 41709284
Phenotypes for gene: SF3B3 were set to Neurodevelopmental disorder, MONDO:0700092, SF3B3-related
Review for gene: SF3B3 was set to GREEN
Added comment: PMID 41709284 reports 24 individuals from 24 families with de novo heterozygous loss-of-function (haploinsufficiency) SF3B3 variants presenting with a syndromic neurodevelopmental disorder characterised by autism, developmental delay, intellectual disability, language and motor delay, multiple congenital anomalies and distinctive facial features. Functional assays in patient-derived fibroblasts demonstrate reduced SF3B3 protein levels, proteasome-mediated degradation and transcriptomic/splicing dysregulation.
Sources: Literature