Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV004961895 | SCV005468781 | pathogenic | Inborn genetic diseases | 2024-10-03 | criteria provided, single submitter | clinical testing | The c.3514C>T (p.Q1172*) alteration, located in exon 15 (coding exon 15) of the PKD1 gene, consists of a C to T substitution at nucleotide position 3514. This changes the amino acid from a glutamine (Q) to a stop codon at amino acid position 1172. This alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. This variant was not reported in population-based cohorts in the Genome Aggregation Database (gnomAD). This variant was reported in multiple individuals with features consistent with PKD1-related polycystic kidney disease (Yilmaz, 2024; Nigro, 2023; Kim, 2019; Xu, 2018). Based on the available evidence, this alteration is classified as pathogenic. |
Department of Pathology and Laboratory Medicine, |
RCV005392905 | SCV006052526 | pathogenic | Polycystic kidney disease, adult type | 2024-10-11 | criteria provided, single submitter | clinical testing |