Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000544000 | SCV000634164 | uncertain significance | Leukocyte adhesion deficiency 1 | 2017-07-30 | criteria provided, single submitter | clinical testing | This variant has not been reported in the literature in individuals with ITGB2-related disease. 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. Algorithms developed to predict the effect of missense changes on protein structure and function do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Benign"; Align-GVGD: "Class C35"). This variant is present in population databases (rs763707922, ExAC 0.001%). This sequence change replaces glutamine with arginine at codon 101 of the ITGB2 protein (p.Gln101Arg). The glutamine residue is highly conserved and there is a small physicochemical difference between glutamine and arginine. |