Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Prevention |
RCV003901486 | SCV004715658 | pathogenic | PKD1-related disorder | 2023-10-31 | no assertion criteria provided | clinical testing | The PKD1 c.1589G>A variant is predicted to result in the amino acid substitution p.Cys530Tyr. This variant has not been reported in a large population database (http://gnomad.broadinstitute.org), indicating this variant is rare. This variant has been reported in individuals with autosomal dominant polycystic kidney disease (ADPKD) (Liu et al. 2015. PubMed ID: 26274329; Simms et al. 2015. PubMed ID: 25340609). The p.Cys530 residue is highly conserved during evolution and other variants affecting flanking highly-conserved codons have been reported to be pathogenic for ADPKD (Human Gene Mutation Database - HGMD). Of note, we have found this variant in the heterozygous state in an individual tested for autosomal dominant polycystic kidney disease (ADPKD) at PreventionGenetics. This variant is interpreted as pathogenic. |