Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV005237017 | SCV005884253 | likely pathogenic | Osteoporosis | 2024-12-10 | criteria provided, single submitter | clinical testing | Variant summary: COL1A2 c.1604G>T (p.Gly535Val) results in a non-conservative amino acid change located in the Collagen triple helix repeat region (Uniprot IPR008160) of the encoded protein sequence. This missense variant disrupts a critical glycine residue at position 1 of a Gly-X-Y repeat in the collagenous domain of COL1A2 protein, and at-least one other missense affecting this glycine residue has been reported in patient(s) with skeletal dysplasia (PMID 34091931, p.Gly535Ala). Five of five in-silico tools predict a damaging effect of the variant on protein function. The variant was absent in 251480 control chromosomes. To our knowledge, no occurrence of c.1604G>T in individuals affected with Osteoporosis and no experimental evidence demonstrating its impact on protein function have been reported. No submitters have cited clinical-significance assessments for this variant to ClinVar. Based on the evidence outlined above, the variant was classified as likely pathogenic. |