Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Victorian Clinical Genetics Services, |
RCV001251464 | SCV001427164 | uncertain significance | Polycystic kidney disease, adult type | 2018-07-12 | criteria provided, single submitter | clinical testing | A heterozygous missense variant, NM_001009944.2(PKD1):c.2473C>T, has been identified in exon 11 of 46 of the PKD1 gene. The variant is predicted to result in a major amino acid change from arginine to tryptophan at position 825 of the protein (NP_001009944.2(PKD1):p.(Arg825Trp)). The arginine at this position has low conservation (100 vertebrates, UCSC), and is not located within a well established functional domain. In silico predictions of pathogenicity for this variant are conflicting (Polyphen, SIFT, CADD, Mutation Taster). The variant is present in the gnomAD database at a frequency of 0.003% (7 heterozygotes, 0 homozygotes), as is an alternative change, p.(Arg825Gln), at a frequency of 0.001% (3 heterozygotes, 0 homozygotes). This variant has not been previously reported in clinical cases. Based on the information available at the time of curation, this variant has been classified as a VARIANT of UNCERTAIN SIGNIFICANCE (VUS). |
Fulgent Genetics, |
RCV001251464 | SCV005643116 | uncertain significance | Polycystic kidney disease, adult type | 2024-04-08 | criteria provided, single submitter | clinical testing |