OPA1 Recombinant Rabbit Monoclonal Antibody [PSH06-39] (HA722673)

UNIPROT #

O60313

PROTEIN NAME

OPA1

SYNONYMS

Dynamin like 120 kDa protein antibody;Dynamin like 120 kDa protein, mitochondrial antibody;Dynamin-like 120 kDa protein, form S1 antibody;FLJ12460 antibody;Juvenile kjer type optic atrophy antibody;KIAA0567 antibody;KJER type antibody;Large GTP binding protein antibody;largeG antibody;MGM1 antibody;Mitochondrial dynamin like 120 kDa protein antibody;Mitochondrial dynamin like GTPase antibody;NPG antibody;NTG antibody;OAK antibody;OPA 1 antibody;opa1 antibody;OPA1 gene antibody;OPA1_HUMAN antibody;Optic atrophy 1 (autosomal dominant) antibody;OPTIC ATROPHY 1 antibody;Optic atrophy 1 gene protein antibody;Optic atrophy 1 homolog (human) antibody;Optic atrophy protein 1 antibody;Optic atrophy protein 1 homolog antibody

SUBCELLULAR LOCATION

Mitochondrion inner membrane.

FUNCTION

This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. The OPA1 protein localizes to the inner mitochondrial membrane, where it regulates mitochondrial fusion and cristae structure. OPA1 mediates mitochondrial fusion in cooperation with mitofusins 1 and 2 and participates in cristae remodeling by the oligomerization of two L-OPA1 and one S-OPA1, which then interact with other protein complexes to alter cristae structure. Its cristae regulating function also contributes to its role in oxidative phosphorylation and apoptosis, as it is required to maintain mitochondrial activity during low-energy substrate availability. Moreover, stabilization of mitochondrial cristae by OPA1 protects against mitochondrial dysfunction, cytochrome c release, and reactive oxygen species production, thus preventing cell death. Mitochondrial SLC25A transporters can detect these low levels and stimulate OPA1 oligomerization, leading to tightening of the cristae, enhanced assembly of ATP synthase, and increased ATP production. Stress from an apoptotic response can interfere with OPA1 oligomerization and prevent mitochondrial fusion.