Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001230596 | SCV001403080 | uncertain significance | Stormorken syndrome; Combined immunodeficiency due to STIM1 deficiency; Myopathy with tubular aggregates | 2022-02-09 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. ClinVar contains an entry for this variant (Variation ID: 957596). This missense change has been observed in individual(s) with clinical features of tubular aggregate myopathy (Invitae). This variant is present in population databases (rs748820994, gnomAD 0.002%). This sequence change replaces asparagine, which is neutral and polar, with serine, which is neutral and polar, at codon 554 of the STIM1 protein (p.Asn554Ser). |
Mayo Clinic Laboratories, |
RCV004792830 | SCV005412278 | uncertain significance | not provided | 2024-01-22 | criteria provided, single submitter | clinical testing | BP4, PM1_supporting, PM2_moderate |