Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV004700183 | SCV005201686 | likely pathogenic | not provided | 2023-11-22 | criteria provided, single submitter | clinical testing | Published functional studies suggest a damaging effect: reduced colony survival upon exposure to ionizing radiation compared to wildtype without kinase activity (PMID: 18634022); Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; This variant is associated with the following publications: (PMID: 22529920, 9450874, 18634022) |
OMIM | RCV000003167 | SCV000023325 | pathogenic | Ataxia-telangiectasia without immunodeficiency | 1998-01-13 | no assertion criteria provided | literature only |