Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000532653 | SCV000640893 | uncertain significance | Nemaline myopathy 2 | 2022-07-30 | criteria provided, single submitter | clinical testing | This sequence change replaces methionine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 3060 of the NEB protein (p.Met3060Val). This variant is present in population databases (rs538524863, gnomAD 0.1%). This variant has not been reported in the literature in individuals affected with NEB-related conditions. ClinVar contains an entry for this variant (Variation ID: 465650). 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: "Not Available"; Align-GVGD: "Class C0"). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. 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. |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004701625 | SCV005203890 | uncertain significance | not specified | 2024-07-02 | criteria provided, single submitter | clinical testing | Variant summary: NEB c.9178A>G (p.Met3060Val) results in a conservative amino acid change in the encoded protein sequence. Three of four in-silico tools predict a benign effect of the variant on protein function. The variant allele was found at a frequency of 0.00038 in 249242 control chromosomes. This frequency is not significantly higher than estimated for a pathogenic variant in NEB causing Nemaline Myopathy 2 (0.00038 vs 0.0035), allowing no conclusion about variant significance. To our knowledge, no occurrence of c.9178A>G in individuals affected with Nemaline Myopathy 2 and no experimental evidence demonstrating its impact on protein function have been reported. ClinVar contains an entry for this variant (Variation ID: 465650). Based on the evidence outlined above, the variant was classified as uncertain significance. |
Natera, |
RCV000532653 | SCV002077173 | uncertain significance | Nemaline myopathy 2 | 2019-11-11 | no assertion criteria provided | clinical testing |