Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV000988866 | SCV001138760 | uncertain significance | Severe early-onset pulmonary alveolar proteinosis due to MARS deficiency | 2019-05-28 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002236000 | SCV002509706 | uncertain significance | Severe early-onset pulmonary alveolar proteinosis due to MARS deficiency; Charcot-Marie-Tooth disease axonal type 2U | 2022-10-17 | criteria provided, single submitter | clinical testing | This variant has not been reported in the literature in individuals affected with MARS-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces glycine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 415 of the MARS protein (p.Gly415Val). ClinVar contains an entry for this variant (Variation ID: 802867). 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 disruptive. 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. |