Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002513108 | SCV003524966 | likely pathogenic | Ehlers-Danlos syndrome, classic type, 1 | 2022-03-13 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt COL5A2 protein function. ClinVar contains an entry for this variant (Variation ID: 17198). This missense change has been observed in individual(s) with Ehlers-Danlos syndrome (PMID: 9783710). It has also been observed to segregate with disease in related individuals. This variant is present in population databases (rs121912930, gnomAD 0.0009%). This sequence change replaces glycine, which is neutral and non-polar, with arginine, which is basic and polar, at codon 1149 of the COL5A2 protein (p.Gly1149Arg). |
OMIM | RCV000018738 | SCV000039021 | pathogenic | Ehlers-Danlos syndrome, classic type | 1998-10-01 | no assertion criteria provided | literature only |