Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV002226143 | SCV002504568 | pathogenic | not provided | 2022-04-23 | criteria provided, single submitter | clinical testing | Not observed in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 28333917) |
3billion | RCV002250800 | SCV002521313 | pathogenic | Congenital contractures of the limbs and face, hypotonia, and developmental delay | 2022-05-22 | criteria provided, single submitter | clinical testing | The variant is not observed in the gnomAD v2.1.1 dataset. It is located in a mutational hot spot and/or well-established functional domain in which established pathogenic variants have been reported. Missense changes are a common disease-causing mechanism. In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.80; 3Cnet: 0.73). Same nucleotide change resulting in same amino acid change has been previously reported to be associated with NALCN related disorder (PMID: 28333917). The variant has been previously reported as de novo in a similarly affected individual (PMID: 28333917). Therefore, this variant is classified as pathogenic according to the recommendation of ACMG/AMP guideline. |
Ce |
RCV002226143 | SCV003917295 | likely pathogenic | not provided | 2023-01-01 | criteria provided, single submitter | clinical testing | NALCN: PM1, PM2, PP2, PP3, PS2:Supporting, PS4:Supporting |