Aliases for OMA1 Gene
External Ids for OMA1 Gene
Previous GeneCards Identifiers for OMA1 Gene
GeneCards Summary for OMA1 Gene
OMA1 (OMA1 Zinc Metallopeptidase) is a Protein Coding gene. Diseases associated with OMA1 include Spinocerebellar Ataxia 28 and 3-Methylglutaconic Aciduria, Type Iii. Among its related pathways are CDK-mediated phosphorylation and removal of Cdc6 and Glucose / Energy Metabolism. Gene Ontology (GO) annotations related to this gene include metalloendopeptidase activity.
UniProtKB/Swiss-Prot Summary for OMA1 Gene
Metalloprotease that is part of the quality control system in the inner membrane of mitochondria. Following stress conditions that induce loss of mitochondrial membrane potential, mediates cleavage of OPA1 at S1 position, leading to OPA1 inactivation and negative regulation of mitochondrial fusion. May also cleave UQCC3 under these conditions. Its role in mitochondrial quality control is essential for regulating lipid metabolism as well as to maintain body temperature and energy expenditure under cold-stress conditions.