Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004547401 | SCV005042973 | uncertain significance | Mitochondrial DNA depletion syndrome 6 (hepatocerebral type) | criteria provided, single submitter | clinical testing | The missense c.265A>Tp.Met89Leu variant in MPV17 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. This variant is reported with the allele frequency of 0.005% in the gnomAD Exomes and novel in 1000 Genomes. The amino acid Met at position 89 is changed to a Leu changing protein sequence and it might alter its composition and physico-chemical properties. The amino acid change p.Met89Leu in MPV17 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The variant is predicted as damaging by SIFT. For these reasons, this variant has been classified as Uncertain Significance. |