Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000478343 | SCV000573101 | pathogenic | not provided | 2017-02-16 | criteria provided, single submitter | clinical testing | The W1157X variant in the SYNGAP1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The W1157X variant is not observed in large population cohorts (Lek et al., 2016; 1000 Genomes Consortium et al., 2015; Exome Variant Server). We interpret W1157X as a pathogenic variant. |
Labcorp Genetics |
RCV003615842 | SCV004537054 | pathogenic | Intellectual disability, autosomal dominant 5 | 2022-12-07 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Trp1157*) in the SYNGAP1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in SYNGAP1 are known to be pathogenic (PMID: 23161826, 23708187, 26989088). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with SYNGAP1-related conditions. ClinVar contains an entry for this variant (Variation ID: 423403). For these reasons, this variant has been classified as Pathogenic. |