Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
NIHR Bioresource Rare Diseases, |
RCV000790359 | SCV000891152 | pathogenic | MYH9-related disorder | 2018-12-12 | criteria provided, single submitter | research | PS4, PP1_strong, PM2, PP4, PP3 |
Labcorp Genetics |
RCV002513293 | SCV003444345 | pathogenic | not provided | 2022-09-06 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (gnomAD no frequency). For these reasons, this variant has been classified as Pathogenic. This variant disrupts the p.Asp1424 amino acid residue in MYH9. Other variant(s) that disrupt this residue have been determined to be pathogenic (PMID: 11159552, 23207509). This suggests that this residue is clinically significant, and that variants that disrupt this residue are likely to be disease-causing. 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: "Tolerated"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). ClinVar contains an entry for this variant (Variation ID: 38966). This missense change has been observed in individuals with MYH9-related disorders (PMID: 11776386, 16098078, 24186861, 26226608). This sequence change replaces aspartic acid, which is acidic and polar, with tyrosine, which is neutral and polar, at codon 1424 of the MYH9 protein (p.Asp1424Tyr). |
Gene |
RCV000032224 | SCV000055830 | not provided | Macrothrombocytopenia and granulocyte inclusions with or without nephritis or sensorineural hearing loss | no assertion provided | literature only | ||
ISTH- |
RCV000032224 | SCV002515483 | pathogenic | Macrothrombocytopenia and granulocyte inclusions with or without nephritis or sensorineural hearing loss | no assertion criteria provided | research |