OMA1: Proteins and Enzymes

OMA1, also known as Metalloendopeptidase OMA1, mitochondrial, has 2 isoforms, a 524 amino acid long isoform that is 60 kDa and a short 486 amino acid isoform that is 56 kDa; widely expressed, with strong expression in the heart, skeletal muscle, kidney and liver; participates in 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. 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. Studies are being performed on the relationship of this protein to amyotrophic lateral sclerosis, lateral sclerosis, childhood medulloblastoma, and medulloblastoma. OMA1 protein involvement has been observed with relation to diet induced thermogenesis, glucose metabolic process, misfolded or incompletely synthesized protein catabolic process, lipid metabolic process, negative regulation of mitochondrial fusion, and cristae formation processes.