Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Sydney Genome Diagnostics, |
RCV001328278 | SCV001449199 | uncertain significance | Atypical hemolytic-uremic syndrome | 2019-04-02 | no assertion criteria provided | clinical testing | This individual is heterozygous for the c.1678G>A variant in the C3 gene, which results in the amino acid substitution of valine to methionine at residue 560, p.(Val560Met). This variant has been reported in the gnomAD browser (http://gnomad.broadinstitute.org) with a very low allele frequency of 0.001% (3 out of 248,706 alleles). To our knowledge, this variant has not been previously reported in the literature or any disease specific databases to be a disease causing variant. In silico analysis of pathogenicity (through Alamut Visual v2.8.1) using PolyPhen2, SIFT and MutationTaster all predict this variant to be a likely benign variant. However, this analysis alone cannot be used to exclude pathogenicity. This variant is considered to be a variant of uncertain clinical significance (VOUS) according to the ACMG guidelines (Evidence used: BP4, PM2). |