Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Illumina Laboratory Services, |
RCV001095774 | SCV001251618 | pathogenic | Spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 2 | 2020-01-09 | criteria provided, single submitter | clinical testing | The SETX c.4853C>G (p.Ser1618Ter) variant is a stop-gained variant that is predicted to result in a premature termination of the protein. A literature search was performed for the gene, cDNA change, and amino acid change. No publications were found based on this search. The p.Ser1618Ter variant is reported at a frequency of 0.000120 in the African population of the Genome Aggregation Database. Based on the predicted truncating nature of the variant, its rarity, identification in a homozygous state and application of the ACMG criteria, the p.Ser1618Ter variant is classified as pathogenic for autosomal recessive ataxia with oculomotor apraxia type 2. |