Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Smedley Team, |
RCV004813558 | SCV005431538 | likely pathogenic | Monogenic diabetes | no assertion criteria provided | case-control | This variant, resulting in a stop-gain in UNC13A (p.Gly44*) that is predicted to lead to NMD (PVS1), was detected in a patient recruited to the 100,000 Genomes Project under the 'diabetes with additional phenotypes suggestive of a monogenic aetiology' specific disease category. In addition, this variant is absent from gnomAD and was classified as PM2 leading to an overall pathogenic classification. UNC13A was identified as a novel disease-gene association for monongenic diabetes in a gene burden study of the 100,000 Genomes Project. |