Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000657783 | SCV000779536 | pathogenic | not provided | 2022-05-19 | criteria provided, single submitter | clinical testing | Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); Has not been previously reported as pathogenic or benign in an affected individual to our knowledge; This variant is associated with the following publications: (PMID: 31589614) |
Labcorp Genetics |
RCV005091889 | SCV005782030 | pathogenic | Intellectual disability, autosomal dominant 1 | 2024-11-19 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg1207*) in the MBD5 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in MBD5 are known to be pathogenic (PMID: 23422940, 23587880). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with MBD5-related conditions. ClinVar contains an entry for this variant (Variation ID: 546027). For these reasons, this variant has been classified as Pathogenic. |