Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003338180 | SCV004047170 | uncertain significance | Autosomal recessive DOPA responsive dystonia | criteria provided, single submitter | clinical testing | The missense variant c.920T>C (p.Val307Ala) in TH gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Val307Ala variant is novel (not in any individuals) in gnomAD Exomes and 1000 Genomes. The amino acid Val at position 307 is changed to a Ala changing protein sequence and it might alter its composition and physico-chemical properties. The amino acid change p.Val307Ala in TH is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance . |