Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV003232018 | SCV003930236 | uncertain significance | not provided | 2023-05-30 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Neuberg Centre For Genomic Medicine, |
RCV004818309 | SCV005438836 | uncertain significance | Polycystic kidney disease, adult type | 2023-07-22 | criteria provided, single submitter | clinical testing | The missense variant c.9715G>A p.Asp3239Asn in the PKD1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. This variant is reported with the allele frequency 0.001% in the gnomAD Exomes. This variant has been reported to the ClinVar database as Uncertain Significance. However, no details are available for independent assessment. The amino acid Aspartic Acid at position 3239 is changed to an Asparagine changing protein sequence and it might alter its composition and physico-chemical properties. Multiple lines of computational evidence Polyphen - Damaging, SIFT - Damaging and MutationTaster - Disease causing predict a damaging effect on protein structure and function for this variant. The amino acid change p.Asp3239Asn in PKD1 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance |
Ambry Genetics | RCV004961246 | SCV005475123 | uncertain significance | Inborn genetic diseases | 2024-09-20 | criteria provided, single submitter | clinical testing | The c.9715G>A (p.D3239N) alteration is located in exon 29 (coding exon 29) of the PKD1 gene. This alteration results from a G to A substitution at nucleotide position 9715, causing the aspartic acid (D) at amino acid position 3239 to be replaced by an asparagine (N). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Fulgent Genetics, |
RCV004818309 | SCV005638630 | uncertain significance | Polycystic kidney disease, adult type | 2024-05-23 | criteria provided, single submitter | clinical testing |