Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV004815184 | SCV005439242 | pathogenic | not provided | 2024-06-10 | criteria provided, single submitter | clinical testing | Identified in a patient with vascular Ehlers-Danlos syndrome in the published literature, however detailed clinical information was not provided (PMID: 24922459); Occurs in the triple helical domain and replaces the glycine in the canonical Gly-X-Y repeat; missense substitution of a canonical glycine residue is expected to disrupt normal protein folding and function, and this is an established mechanism of disease (HGMD); Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 24922459) |
Collagen Diagnostic Laboratory, |
RCV000087370 | SCV000120252 | pathogenic | Ehlers-Danlos syndrome, type 4 | no assertion criteria provided | clinical testing |