Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000521247 | SCV000617031 | pathogenic | not provided | 2024-02-22 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); Missense variants in this gene are a common cause of disease and they are underrepresented in the general population; In silico analysis supports that this missense variant does not alter protein structure/function; This variant is associated with the following publications: (PMID: 33334799, 38348958) |
Department of Clinical Genetics, |
RCV002226715 | SCV002505712 | likely pathogenic | Baraitser-Winter syndrome 1 | 2021-08-01 | criteria provided, single submitter | clinical testing | |
3billion | RCV002226715 | SCV002572794 | pathogenic | Baraitser-Winter syndrome 1 | 2022-09-01 | criteria provided, single submitter | clinical testing | The variant is not observed in the gnomAD v2.1.1 dataset. Missense changes are a common disease-causing mechanism. In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.64; 3Cnet: 0.82). Same nucleotide change resulting in same amino acid change has been previously reported as pathogenic/likely pathogenic with strong evidence (ClinVar ID: VCV000449190). The variant has been previously reported as assumed (i.e. paternity and maternity not confirmed) de novo in at least one similarly affected unrelated individual (3billion dataset). Therefore, this variant is classified as Pathogenic according to the recommendation of ACMG/AMP guideline. |
Institute for Medical Genetics and Human Genetics, |
RCV002226715 | SCV004037179 | pathogenic | Baraitser-Winter syndrome 1 | criteria provided, single submitter | not provided |