Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Concord Molecular Medicine Laboratory, |
RCV004800940 | SCV005414526 | uncertain significance | Malignant hyperthermia, susceptibility to, 1 | 2024-11-26 | criteria provided, single submitter | clinical testing | This variant was detected in an individual with history of malignant hyperthermia, as well as proximal and central muscle weakness. The variant is absent from control population database (gnomAD v.4.1.0). It is located at the C-terminal region but distal to the known hotspot region as defined by ClinGen VCEP (PMID: 33767344). In silico analysis suggests a pathogenic effect (REVEL 0.87). |