Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Igenomix - |
RCV004691649 | SCV005187252 | uncertain significance | Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A1 | criteria provided, single submitter | clinical testing | The POMT1 variant (NM_001077365.2:c.2001C>G, p.Cys667Trp) replaces the amino acid Cysteine with Trypotophan. The in silico tools predict a moderately deleterious affect of the variant on the protein. This variant is absent in the gnomAD v4.1.0 (PM2). This variant has not been reported in the literature in individuals affected with POMT1-related conditions. Based on the evidence outlined above, the variant was classified as variant of uncertain significance (PM2, PP3). The variant was identified in the heterozygous state through carrier screening. |