Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000721148 | SCV003442299 | uncertain significance | Ectodermal dysplasia and immunodeficiency 2 | 2022-09-28 | criteria provided, single submitter | clinical testing | ClinVar contains an entry for this variant (Variation ID: 590307). 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. Experimental studies are conflicting or provide insufficient evidence to determine the effect of this variant on NFKBIA function (PMID: 28629746). 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. This missense change has been observed in individual(s) with autosomal dominant ectodermal dysplasia (PMID: 23708964). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces methionine, which is neutral and non-polar, with lysine, which is basic and polar, at codon 37 of the NFKBIA protein (p.Met37Lys). |
OMIM | RCV000721148 | SCV000852031 | pathogenic | Ectodermal dysplasia and immunodeficiency 2 | 2018-11-15 | no assertion criteria provided | literature only |