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Autoinflammatory Disorders v3.14 Zornitza Stark Added reviews for gene DNAJC17 from panel Autoinflammatory Disorders
Autoinflammatory Disorders v3.13 DNAJC17 Zornitza Stark Marked gene: DNAJC17 as ready
Autoinflammatory Disorders v3.13 DNAJC17 Zornitza Stark Gene: dnajc17 has been classified as Amber List (Moderate Evidence).
Autoinflammatory Disorders v3.13 DNAJC17 Zornitza Stark Phenotypes for gene: DNAJC17 were changed from Combined Immune deficiency; retinopathy; autoinflammation to Inborn error of immunity, MONDO:0003778, DNAJC17-related
Autoinflammatory Disorders v3.12 DNAJC17 Zornitza Stark Classified gene: DNAJC17 as Amber List (moderate evidence)
Autoinflammatory Disorders v3.12 DNAJC17 Zornitza Stark Gene: dnajc17 has been classified as Amber List (Moderate Evidence).
Autoinflammatory Disorders v3.11 DNAJC17 Zornitza Stark reviewed gene: DNAJC17: Rating: AMBER; Mode of pathogenicity: None; Publications: ; Phenotypes: Inborn error of immunity, MONDO:0003778, DNAJC17-related; Mode of inheritance: BIALLELIC, autosomal or pseudoautosomal
Autoinflammatory Disorders v3.11 DNAJC17 Peter McNaughton gene: DNAJC17 was added
gene: DNAJC17 was added to Autoinflammatory Disorders. Sources: Literature
Mode of inheritance for gene: DNAJC17 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: DNAJC17 were set to PMID: 42495638
Phenotypes for gene: DNAJC17 were set to Combined Immune deficiency; retinopathy; autoinflammation
Review for gene: DNAJC17 was set to GREEN
Added comment: 3 patients from 2x unrelated families with homozygous variant (c.681G>A; p.Ala227=) with molecular characterisation suggesting exon skipping and reduced but not absent DNAJC17
mRNA and protein expression supporting the hypothesis of hypomorphic
rather than null function.
Sources: Literature