OPA1 Recombinant Rabbit Monoclonal Antibody [JM81-35] (ET1705-9)

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

SEQUENCE SIMILARITIES

Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family.

TISSUE SPECIFICITY

Highly expressed in retina. Also expressed in brain, testis, heart and skeletal muscle. Isoform 1 expressed in retina, skeletal muscle, heart, lung, ovary, colon, thyroid gland, leukocytes and fetal brain. Isoform 2 expressed in colon, liver, kidney, thyroid gland and leukocytes. Low levels of all isoforms expressed in a variety of tissues.

POST-TRANSLATIONAL MODIFICATION

PARL-dependent proteolytic processing releases an antiapoptotic soluble form not required for mitochondrial fusion. Cleaved by OMA1 at position S1 following stress conditions.; [Isoform 2]: Cleavage at position S2 is mediated by YME1L. Cleavage may occur in the sequence motif Leu-Gln-Gln-Gln-Ile-Gln (LQQQIQ).; [Isoform 3]: Cleavage at position S2 is mediated by YME1L. Cleavage may occur in the sequence motif Leu-Gln-Gln-Gln-Ile-Gln (LQQQIQ).; [Isoform 4]: Cleavage at position S2 is mediated by YME1L. Cleavage may occur in the sequence motif Leu-Gln-Gln-Gln-Ile-Gln (LQQQIQ).

SUBCELLULAR LOCATION

Mitochondrion inner membrane. Mitochondrion intermembrane space.

FUNCTION

Dynamin-related GTPase that is essential for normal mitochondrial morphology by regulating the equilibrium between mitochondrial fusion and mitochondrial fission .Coexpression of isoform 1 with shorter alternative products is required for optimal activity in promoting mitochondrial fusion.Binds lipid membranes enriched in negatively charged phospholipids, such as cardiolipin, and promotes membrane tubulation.The intrinsic GTPase activity is low, and is strongly increased by interaction with lipid membranes.Plays a role in remodeling cristae and the release of cytochrome c during apoptosis.Proteolytic processing in response to intrinsic apoptotic signals may lead to disassembly of OPA1 oligomers and release of the caspase activator cytochrome C (CYCS) into the mitochondrial intermembrane space. Plays a role in mitochondrial genome maintenance.