Recombinant Human Metalloendopeptidase OMA1, mitochondrial (OMA1), Truncated

Uniprot Id

Q96E52

Target Species

Human

Target Name

OMA1

Target Full Name

Metalloendopeptidase OMA1, mitochondrial

Target Function

Metalloprotease that is part of the quality control system in the inner membrane of mitochondria. Activated in response to various mitochondrial stress, leading to the proteolytic cleavage of target proteins, such as OPA1, UQCC3 and DELE1. 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. Also acts as a regulator of apoptosis: upon BAK and BAX aggregation, mediates cleavage of OPA1, leading to the remodeling of mitochondrial cristae and allowing the release of cytochrome c from mitochondrial cristae. In depolarized mitochondria, may also act as a backup protease for PINK1 by mediating PINK1 cleavage and promoting its subsequent degradation by the proteasome. May also cleave UQCC3 in response to mitochondrial depolarization. Also acts as an activator of the integrated stress response (ISR): in response to mitochondrial stress, mediates cleavage of DELE1 to generate the processed form of DELE1 (S-DELE1), which translocates to the cytosol and activates EIF2AK1/HRI to trigger the ISR. 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. Binds cardiolipin, possibly regulating its protein turnover. Required for the stability of the respiratory supercomplexes.

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein.

Target Protein Families

Peptidase M48 family

Target Tissue Specificity

Widely expressed, with strong expression in the heart, skeletal muscle, kidney and liver.

Target Synonyms

OMA1; MPRP1; Metalloendopeptidase OMA1, mitochondrial; Metalloprotease-related protein 1; MPRP-1; Overlapping with the m-AAA protease 1 homolog

Target Background

Enables metalloendopeptidase activity. Involved in several processes, including HRI-mediated signaling; proteolysis; and regulation of mitochondrion organization. Located in mitochondrial inner membrane.