Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Santos- |
RCV000770774 | SCV000899106 | likely pathogenic | Autosomal dominant nonsyndromic hearing loss 4A | 2019-01-17 | criteria provided, single submitter | research | |
Labcorp Genetics |
RCV001855975 | SCV002193344 | uncertain significance | not provided | 2022-07-18 | criteria provided, single submitter | clinical testing | ClinVar contains an entry for this variant (Variation ID: 626217). 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 disruptive. This variant is also known as c.2971G>A (p.Glu991Lys). This missense change has been observed in individual(s) with clinical features of MYH14-related conditions (PMID: 30828794). The frequency data for this variant in the population databases is considered unreliable, as metrics indicate poor data quality at this position in the gnomAD database. This sequence change replaces glutamic acid, which is acidic and polar, with lysine, which is basic and polar, at codon 950 of the MYH14 protein (p.Glu950Lys). |
Gene |
RCV001855975 | SCV002496259 | uncertain significance | not provided | 2023-05-10 | criteria provided, single submitter | clinical testing | Identified in a patient with congenital bilateral profound sensorineural hearing loss in published literature (Truong et al., 2019); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 30828794) |