Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001040101 | SCV001203657 | uncertain significance | Pyruvate dehydrogenase E2 deficiency | 2019-11-26 | 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 (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated, but these predictions have not been confirmed by published functional studies and their clinical significance is uncertain. This variant has not been reported in the literature in individuals with DLAT-related conditions. This variant is present in population databases (rs782810132, ExAC 0.02%). This sequence change replaces phenylalanine with serine at codon 571 of the DLAT protein (p.Phe571Ser). The phenylalanine residue is moderately conserved and there is a large physicochemical difference between phenylalanine and serine. |