Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
General Neurology and Ataxia Unit, |
RCV001787700 | SCV002031293 | likely pathogenic | Spinocerebellar ataxia type 28 | 2021-11-01 | criteria provided, single submitter | clinical testing | NM_006796.3(AFG3L2):c.2062C>A;p.(Pro688Thr) is a missense variant located within the peptidase-M41 domain of the protein, a common site of missense variants that cause autosomal dominant spinocerebellar ataxia (PMID: 20725928). This variant is absent from population databases (gnomAD, ABraOM). Previously, variants in the same amino acid where reported as disease-causing (p.P688A - PMID: 28444220; p.P688S - ClinVar Variation ID: 987295). In silico prediction tools agree on disease-causing effect of the protein (REVEL, MetaLR, MetaSVM, MetaRNN). |
Gene |
RCV001787700 | SCV002562251 | not provided | Spinocerebellar ataxia type 28 | no assertion provided | literature only |