Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
NIHR Bioresource Rare Diseases, |
RCV001027557 | SCV001190127 | pathogenic | Combined immunodeficiency | 2019-01-01 | criteria provided, single submitter | research | |
NIHR Bioresource Rare Diseases, |
RCV001027558 | SCV001190128 | pathogenic | Immunodeficiency | 2019-01-01 | criteria provided, single submitter | research | |
Labcorp Genetics |
RCV001230251 | SCV001402725 | uncertain significance | Lymphoproliferative syndrome 2 | 2022-10-05 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with cysteine, which is neutral and slightly polar, at codon 107 of the CD27 protein (p.Arg107Cys). This variant is present in population databases (rs371761387, gnomAD 0.01%). This missense change has been observed in individual(s) with clinical features of CD27 deficiency (PMID: 25843314, 32499645). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. ClinVar contains an entry for this variant (Variation ID: 827695). 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 not expected to disrupt CD27 protein function. 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. |