Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV002511097 | SCV002820794 | pathogenic | not provided | 2025-03-17 | criteria provided, single submitter | clinical testing | In-frame deletion of 3 of amino acidsin a non-repeat region predicted to critically alter the protein; Occurs in the triple helical domain and is predicted to remove canonical Gly-X-Y repeat units; an in-frame deletion variant in this region is expected to disrupt normal protein folding and function, and this is an established mechanism of disease; In silico analysis supports a deleterious effect on protein structure/function; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 34416374) |
Wang |
RCV001822886 | SCV001762459 | likely pathogenic | Stickler syndrome type 1 | 2021-07-01 | no assertion criteria provided | clinical testing |