Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001033347 | SCV001196654 | pathogenic | Stormorken syndrome; Combined immunodeficiency due to STIM1 deficiency; Myopathy with tubular aggregates | 2019-12-08 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in STIM1 are known to be pathogenic (PMID: 19420366, 20876309). This variant has not been reported in the literature in individuals with STIM1-related conditions. This variant is an out-of-frame deletion of the genomic region encompassing exon 5 of the STIM1 gene. This is expected to create a premature translational stop signal and result in an absent or disrupted protein product. |