Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Sydney Children's Hospital, |
RCV000190424 | SCV000191919 | pathogenic | Brunner syndrome | 2014-11-10 | criteria provided, single submitter | clinical testing | Phenotype consistent with described MAOA deficiency. Functional studies (bioamine levels in blood and urine) consistent with MAOA deficiency. Predicted Pathogenic PROVEAN, SIFT, Mutation Taster. Not listed Exac Database. |
Gene |
RCV002293426 | SCV002586811 | likely pathogenic | not provided | 2022-04-28 | criteria provided, single submitter | clinical testing | 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: 25807999, 11700166) |
OMIM | RCV000190424 | SCV000747034 | pathogenic | Brunner syndrome | 2015-03-23 | no assertion criteria provided | literature only |