Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Biology Laboratory, |
RCV001281146 | SCV001425151 | likely pathogenic | Polycystic kidney disease, adult type | 2020-02-01 | criteria provided, single submitter | research | |
Fulgent Genetics, |
RCV001281146 | SCV005640578 | uncertain significance | Polycystic kidney disease, adult type | 2024-06-03 | criteria provided, single submitter | clinical testing | |
Department of Pathology and Laboratory Medicine, |
RCV001292495 | SCV001480611 | uncertain significance | Polycystic kidney disease | no assertion criteria provided | clinical testing | PKD1, EXON05, c.1154T>C, p.Leu385Pro, Heterozygous, Uncertain SignificancernThe PKD1 p.Leu385Pro variant was not identified in the literature nor was it identified in the following databases: dbSNP, ClinVar, LOVD 3.0, ADPKD Mutation Database, or PKD1-LOVD. The variant was not identified in the following control databases: the Exome Aggregation Consortium (August 8th 2016), or the Genome Aggregation Database (Feb 27, 2017). The p.Leu385 residue is conserved in mammals and computational analyses (PolyPhen-2, SIFT, AlignGVGD, BLOSUM, MutationTaster) provide inconsistent predictions regarding the impact to the protein; this information is not very predictive of pathogenicity. The variant occurs outside of the splicing consensus sequence and 1 of 4 in silico or computational prediction software programs (SpliceSiteFinder, MaxEntScan, NNSPLICE, GeneSplicer) predict a greater than 10% difference in splicing; this is not very predictive of pathogenicity. In summary, based on the above information the clinical significance of this variant cannot be determined with certainty at this time. This variant is classified as a variant of uncertain significance. Assessment Date: 2019/06/26. |