Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV001823704 | SCV002073340 | uncertain significance | 3-hydroxy-3-methylglutaryl-CoA synthase deficiency | criteria provided, single submitter | clinical testing | The splice region variant c.559+5G>A in HMGCS2 (NM_005518.4) has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The c.559+5G>A variant is novel (not in any individuals) in gnomAD Exomes and is novel (not in any individuals) in 1000 Genomes. In silico splicing predictions are contradictory. The nucleotide c.559+5G>A in HMGCS2 is predicted conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance. |