Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002543278 | SCV003473208 | uncertain significance | not provided | 2022-03-25 | 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. Experimental studies have shown that this missense change affects LIG1 function (PMID: 30395541, 32914844, 33444456, 33600799). 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 disruptive. This missense change has been observed in individuals with DNA ligase I deficiency (PMID: 30395541). This variant is present in population databases (rs34087182, gnomAD 0.004%). This sequence change replaces arginine, which is basic and polar, with leucine, which is neutral and non-polar, at codon 641 of the LIG1 protein (p.Arg641Leu). |
OMIM | RCV001843698 | SCV002102820 | pathogenic | Immunodeficiency 96 | 2022-03-07 | no assertion criteria provided | literature only |