Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center for Genomic Medicine, |
RCV004765416 | SCV005375233 | pathogenic | Childhood encephalopathy due to thiamine pyrophosphokinase deficiency | 2024-10-13 | criteria provided, single submitter | clinical testing | This variant (GRCh38; NM_022445.4:c.355-2A>G) results in a Splice Acceptor site mutation in the TPK1 gene. To our knowledge this variant not been previously curated or reported in public Database. Not observed at significant frequency in large population cohorts (gnomAD) This variant has a strong Conservation score. In-silico analysis supports that this splice acceptor site variant is pathogenic. This alteration is interpreted as disease-causing mutation, a commonly known mechanism for disease. |