Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001940484 | SCV002187514 | benign | Amyotrophic lateral sclerosis type 4; Spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 2 | 2023-05-08 | criteria provided, single submitter | clinical testing | |
Neuberg Centre For Genomic Medicine, |
RCV003339824 | SCV004048557 | uncertain significance | Amyotrophic lateral sclerosis type 4 | criteria provided, single submitter | clinical testing | The c.5302A>T(p.Asn1768Tyr) missense variant in SETX gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Asn1768Tyr variant is reported with the allele frequency (0.001%) in the gnomAD Exomes and is novel (not in any individuals) in 1000 Genomes. The amino acid Asn at position 1768 is changed to a Tyr changing protein sequence and it might alter its composition and physico-chemical properties. In silico tools predict the variant to be tolerated. The residue is conserved across species. The amino acid change p.Asn1768Tyr in SETX is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Variant of Uncertain Significance (VUS). |