Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Broad Center for Mendelian Genomics, |
RCV000785891 | SCV000924467 | likely pathogenic | Mitochondrial DNA depletion syndrome 12A (cardiomyopathic type), autosomal dominant | 2018-06-15 | criteria provided, single submitter | research | The heterozygous p.Arg80Gly variant was identified by our study in one individual with mitochondrial DNA depletion syndrome. Trio exome analysis showed this variant to be de novo. This variant was absent from large population studies, and the Arginine (Arg) at position 80 is highly conserved in mammals and evolutionarily distant species, supporting that a change at this position may not be tolerated. In summary, although additional studies are required to fully establish its pathogenicity, this variant is likely pathogenic. |