Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV003313583 | SCV004012569 | likely pathogenic | not provided | 2023-01-05 | criteria provided, single submitter | clinical testing | Reported as c.175_176insA, zygosity unknown, in an individual with retinitis pigmentosa, cerebellar ataxia, cognitive impairment, axonal polyneuropathy with demyelinating features, and actional tremor in the published literature (Vural et al., 2013); Frameshift variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 23775883) |