Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratoire de Génétique Moléculaire, |
RCV001281630 | SCV001468962 | pathogenic | not provided | criteria provided, single submitter | clinical testing | ||
Labcorp Genetics |
RCV001871641 | SCV002162505 | pathogenic | Intellectual disability, autosomal dominant 5 | 2022-07-26 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gly1061Trpfs*92) 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). For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 992861). This premature translational stop signal has been observed in individual(s) with SYNGAP1-related disorders (PMID: 30577886, 33639450). In at least one individual the variant was observed to be de novo. |