Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002233886 | SCV000937193 | pathogenic | Stormorken syndrome; Combined immunodeficiency due to STIM1 deficiency; Myopathy with tubular aggregates | 2018-12-25 | criteria provided, single submitter | clinical testing | This variant has not been reported in the literature in individuals with STIM1-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Asn234Leufs*19) in the STIM1 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in STIM1 are known to be pathogenic (PMID: 19420366, 20876309). For these reasons, this variant has been classified as Pathogenic. |
Genome |
RCV003483726 | SCV004228518 | not provided | Myopathy, tubular aggregate, 1; Stormorken syndrome; Combined immunodeficiency due to STIM1 deficiency | no assertion provided | phenotyping only | Variant interpreted as Pathogenic and reported on 01-05-2019 by Lab Invitae. GenomeConnect-Invitae Patient Insights Network assertions are reported exactly as they appear on the patient-provided report from the testing laboratory. Registry team members make no attempt to reinterpret the clinical significance of the variant. Phenotypic details are available under supporting information. |