Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002020532 | SCV002306614 | uncertain significance | X-linked severe congenital neutropenia; Thrombocytopenia 1; Wiskott-Aldrich syndrome | 2020-11-06 | criteria provided, single submitter | clinical testing | This sequence change replaces glutamine with arginine at codon 99 of the WAS protein (p.Gln99Arg). The glutamine residue is highly conserved and there is a small physicochemical difference between glutamine and arginine. 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 variant does not substantially affect WAS protein function (PMID: 19817875). This variant has been observed in individual(s) with clinical features of Wiskott-Aldrich Syndrome (PMID: 8931701). This variant is not present in population databases (ExAC no frequency). |