Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Centre for Inherited Metabolic Diseases, |
RCV001358678 | SCV001554479 | likely pathogenic | Spinocerebellar ataxia type 29 | 2021-04-07 | criteria provided, single submitter | clinical testing | |
Clinical Genomics Laboratory, |
RCV001358678 | SCV005045129 | uncertain significance | Spinocerebellar ataxia type 29 | 2024-03-22 | criteria provided, single submitter | clinical testing | The ITPR1 c.755C>T (p.Thr252Ile) variant, to our knowledge, has not been reported in the medical literature and is absent from the general population (gnomAD v.2.1.1), indicating it is not a common variant. Computational predictors indicate that the variant is damaging, evidence that correlates with impact to ITPR1 function. This variant has been reported in the ClinVar database as a germline likely pathogenic variant by one submitter. Due to limited information, and based on ACMG/AMP guidelines for variant interpretation (Richards S et al., PMID: 25741868), the clinical significance of this variant is uncertain at this time. |