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Mendeliome v2.513 XXYLT1 Zornitza Stark Marked gene: XXYLT1 as ready
Mendeliome v2.513 XXYLT1 Zornitza Stark Gene: xxylt1 has been classified as Amber List (Moderate Evidence).
Mendeliome v2.513 XXYLT1 Zornitza Stark Classified gene: XXYLT1 as Amber List (moderate evidence)
Mendeliome v2.513 XXYLT1 Zornitza Stark Gene: xxylt1 has been classified as Amber List (Moderate Evidence).
Mendeliome v2.512 XXYLT1 Zornitza Stark gene: XXYLT1 was added
gene: XXYLT1 was added to Mendeliome. Sources: Literature
founder tags were added to gene: XXYLT1.
Mode of inheritance for gene: XXYLT1 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: XXYLT1 were set to 42530953
Phenotypes for gene: XXYLT1 were set to Inherited retinal dystrophy, MONDO:0019118, XXYLT1-related
Review for gene: XXYLT1 was set to AMBER
Added comment: PMID 42530953 reports 7 individuals from 5 families with biallelic loss-of-function and missense variants in XXYLT1 presenting with inherited retinal dystrophy (cone‑rod or macular dystrophy). Homozygous c.505‑1G>C splice‑site variant found in four Finnish families (founder allele) and homozygous c.766G>A missense variant in a consanguineous UK family. Phenotypes included visual deterioration, cystoid macular oedema and schisis‑like macular changes; RNA splicing assays show exon 2 skipping for the recurrent c.505‑1G>C founder variant and Xylt1 knockout mice recapitulated retinal abnormalities.
Sources: Literature