Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000523094 | SCV000619925 | likely pathogenic | not provided | 2017-08-10 | criteria provided, single submitter | clinical testing | A variant that is likely pathogenic has been identified in the PCDH19 gene. The Y490S variant has not been published as a pathogenic variant, nor has it been reported as a benign variant to our knowledge. TheY490S variant is not observed in large population cohorts (Lek et al., 2016; 1000 Genomes Consortium et al., 2015; Exome Variant Server). The Y490S variant is a semi-conservative amino acid substitution, which may impact secondary protein structure as these residues differ in some properties. This substitution occurs at a position that is conserved across species, and in silico analysis predicts this variant is probably damaging to the protein structure/function. Therefore, we interpret Y490S as a likely pathogenic variant. |
Neurogenetics Research Program, |
RCV001199428 | SCV001147062 | likely pathogenic | Glycine encephalopathy; Developmental and epileptic encephalopathy, 9 | 2019-12-20 | no assertion criteria provided | research |