Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Cavalleri Lab, |
RCV001095560 | SCV001251191 | likely pathogenic | Polycystic kidney disease, adult type | 2020-02-05 | criteria provided, single submitter | research | PM2, PM4, PP3, PP4 |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV005408693 | SCV006071976 | likely pathogenic | PKD1-Biallelic Autosomal Recessive Polycystic Kidney Disease | 2025-03-21 | criteria provided, single submitter | clinical testing | Variant summary: PKD1 c.7303_7317del15 (p.Arg2435_Arg2439del) results in an in-frame deletion that is predicted to remove 5 amino acids from the encoded protein. The variant was absent in 226388 control chromosomes. c.7303_7317del15 has been reported in the heterozygous state in the literature in multiple individuals affected with autosomal dominant polycystic kidney disease (example, Elhassan_ 2024, Lindemann_ 2023, Benson_ 2021, Rossetti_ 2007, Audrezet_ 2012). These data indicate that the variant is likely to be associated with disease. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publications have been ascertained in the context of this evaluation (PMID: 33454723, 38481516, 36938073, 17582161, 22508176). ClinVar contains an entry for this variant (Variation ID: 873340). Based on the evidence outlined above, the variant was classified as likely pathogenic. |