Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002009719 | SCV002264411 | uncertain significance | Bailey-Bloch congenital myopathy | 2021-04-09 | 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. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). This variant has not been reported in the literature in individuals with STAC3-related conditions. This variant is present in population databases (rs754352201, ExAC 0.01%). This sequence change replaces isoleucine with methionine at codon 333 of the STAC3 protein (p.Ile333Met). The isoleucine residue is highly conserved and there is a small physicochemical difference between isoleucine and methionine. |