Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genomic Medicine Center of Excellence, |
RCV000171294 | SCV000221491 | likely pathogenic | not provided | criteria provided, single submitter | research | ||
Gene |
RCV000171294 | SCV002513645 | pathogenic | not provided | 2022-05-11 | criteria provided, single submitter | clinical testing | Reported as homozygous in two siblings of Saudi ancestry with Alstrom syndrome who were also homozygous for a missense variant in ALMS1; familial studies suggest the two variants may be present on the same allele (in cis) in each parent (Kamal et al., 2020); Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); Also known as p.(S909*) and p.(S908*) using alternate nomenclature; This variant is associated with the following publications: (PMID: Gosadi2021, 35112413, 30488743, 33981653, 31889847) |