Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Myriad Genetics, |
RCV001264018 | SCV001442118 | likely pathogenic | Autosomal recessive limb-girdle muscular dystrophy type 2A | 2020-02-28 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001264018 | SCV002234642 | pathogenic | Autosomal recessive limb-girdle muscular dystrophy type 2A | 2021-10-03 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 984013). This variant has not been reported in the literature in individuals affected with CAPN3-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Trp721*) in the CAPN3 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CAPN3 are known to be pathogenic (PMID: 10330340, 15689361). |