Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV002250242 | SCV002518872 | pathogenic | Infantile neuroaxonal dystrophy | 2022-05-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002250242 | SCV003444487 | uncertain significance | Infantile neuroaxonal dystrophy | 2022-08-25 | criteria provided, single submitter | clinical testing | This missense change has been observed in individuals with clinical features of PLA2G6-related conditions (PMID: 27516098; Invitae). ClinVar contains an entry for this variant (Variation ID: 1686075). 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. 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. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces lysine, which is basic and polar, with glutamic acid, which is acidic and polar, at codon 678 of the PLA2G6 protein (p.Lys678Glu). |