Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV002274578 | SCV002559384 | uncertain significance | not provided | 2024-10-02 | criteria provided, single submitter | clinical testing | Has not been previously published as pathogenic or benign to our knowledge; Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis indicates that this missense variant does not alter protein structure/function; Not located in the triple helical region, where the majority of pathogenic missense variants occur (PMID: 22696272; HGMD); This variant is associated with the following publications: (PMID: 22696272) |
Ambry Genetics | RCV002337424 | SCV002645380 | uncertain significance | Familial thoracic aortic aneurysm and aortic dissection | 2024-07-02 | criteria provided, single submitter | clinical testing | The p.Q1748R variant (also known as c.5243A>G), located in coding exon 65 of the COL5A1 gene, results from an A to G substitution at nucleotide position 5243. The glutamine at codon 1748 is replaced by arginine, an amino acid with highly similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Based on the available evidence, the clinical significance of this variant remains unclear. |