Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV004723263 | SCV005333291 | likely pathogenic | not provided | 2023-11-06 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Pediatric Infectious Diseases and Immunodeficiencies Unit |
RCV003493994 | SCV004023239 | uncertain significance | Common variable immunodeficiency | no assertion criteria provided | clinical testing | This variant was found in an adult female patient with common variable immunodeficiency (CVID). The variant is absent from large population studies and there is no evidence of its functional effect. It is located in the kinase domain of the protein, which contains other pathogenic variants associated with X-linked agammaglobulinemia (XLA). We consider the c.1787T>C a Variant of Uncertain Significance. |