Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003340723 | SCV004047394 | uncertain significance | Peroxisome biogenesis disorder 4B | criteria provided, single submitter | clinical testing | The missense variant c.1183G>A (p.Gly395Arg) in PEX6 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Gly395Arg variant is novel (not in any individuals) in 1000 Genomes and allele frequency of 0.0003977% is reported in gnomAD. The amino acid Gly at position 395 is changed to a Arg changing protein sequence and it might alter its composition and physico-chemical properties. The amino acid change p.Gly395Arg in PEX6 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance. |