Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004587708 | SCV005076240 | pathogenic | Spinocerebellar ataxia type 28 | 2024-04-29 | criteria provided, single submitter | clinical testing | Variant summary: AFG3L2 c.1651C>T (p.Arg551X) results in a premature termination codon, predicted to cause a truncation of the encoded protein or absence of the protein due to nonsense mediated decay, which are commonly known mechanisms for disease. The variant allele was found at a frequency of 1.2e-05 in 251414 control chromosomes. To our knowledge, no occurrence of c.1651C>T in individuals affected with Spinocerebellar Ataxia Type 28 or other AFG3L2-related disorders and no experimental evidence demonstrating its impact on protein function have been reported. No submitters have cited clinical-significance assessments for this variant to ClinVar. Based on the evidence outlined above, the variant was classified as pathogenic. |