Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000019380 | SCV002203321 | pathogenic | Brody myopathy | 2021-09-17 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This sequence change creates a premature translational stop signal (p.Arg198*) in the ATP2A1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in ATP2A1 are known to be pathogenic (PMID: 8841193, 10914677, 23911890). This variant is not present in population databases (ExAC no frequency). This premature translational stop signal has been observed in individual(s) with Brody myopathy (PMID: 8841193). It has also been observed to segregate with disease in related individuals. ClinVar contains an entry for this variant (Variation ID: 17802). |
OMIM | RCV000019380 | SCV000039670 | pathogenic | Brody myopathy | 1996-10-01 | no assertion criteria provided | literature only |