Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000528387 | SCV000654237 | uncertain significance | Nemaline myopathy 8 | 2017-01-23 | criteria provided, single submitter | clinical testing | In summary, this variant is a novel missense change with uncertain impact on protein function. 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: "Possibly Damaging"; Align-GVGD: "Class C0"). This variant is not present in population databases (ExAC no frequency) and has not been reported in the literature in individuals with a KLHL40-related disease. This sequence change replaces valine with phenylalanine at codon 432 of the KLHL40 protein (p.Val432Phe). The valine residue is moderately conserved and there is a small physicochemical difference between valine and phenylalanine. |