Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
NIHR Bioresource Rare Diseases, |
RCV001027631 | SCV001190203 | uncertain significance | Inherited Immunodeficiency Diseases | 2019-01-01 | criteria provided, single submitter | research | |
Kasturba Medical College, |
RCV001805975 | SCV002053785 | likely pathogenic | STAT3-related early-onset multisystem autoimmune disease | criteria provided, single submitter | clinical testing | ||
Labcorp Genetics |
RCV002552425 | SCV003472616 | likely pathogenic | Hyper-IgE recurrent infection syndrome 1, autosomal dominant; STAT3 gain of function | 2022-09-13 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. This variant disrupts the p.Met329 amino acid residue in STAT3. Other variant(s) that disrupt this residue have been determined to be pathogenic (PMID: 33365035, 33717144). This suggests that this residue is clinically significant, and that variants that disrupt this residue are likely to be disease-causing. Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt STAT3 protein function. ClinVar contains an entry for this variant (Variation ID: 827744). This missense change has been observed in individual(s) with STAT3-related conditions (PMID: 30443250, 32499645). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces methionine, which is neutral and non-polar, with arginine, which is basic and polar, at codon 329 of the STAT3 protein (p.Met329Arg). |