OPA1 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. Dynamin-like 120 kDa protein, form S1: Inactive form produced by cleavage at S1 position by OMA1 following stress conditions that induce loss of mitochondrial membrane potential, leading to negative regulation of mitochondrial fusion. Isoforms that contain the alternative exon 4b (present in isoform 4 and isoform 5) are required for mitochondrial genome maintenance, possibly by anchoring the mitochondrial nucleoids to the inner mitochondrial membrane. Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. 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. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.

Protein type: Apoptosis; EC 3.6.5.5; Hydrolase; Membrane protein, integral; Mitochondrial

Chromosomal Location of human Ortholog: 3q29

Cellular Component: axon cytoplasm; cytoplasm; cytosol; dendrite; microtubule; mitochondrial crista; mitochondrial inner membrane; mitochondrial intermembrane space; mitochondrial membrane; mitochondrial outer membrane; mitochondrion; nucleoplasm

Molecular Function: cardiolipin binding; GTP binding; GTPase activity; magnesium ion binding; microtubule binding; phosphatidic acid binding; protein binding

Biological Process: apoptotic process; axonal transport of mitochondrion; cellular senescence; GTP metabolic process; inner mitochondrial membrane organization; membrane tubulation; mitochondrial fission; mitochondrial fusion; mitochondrial genome maintenance; mitochondrion organization; negative regulation of apoptotic process; negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway; negative regulation of release of cytochrome c from mitochondria; neural tube closure; protein complex oligomerization; visual perception

Disease: Behr Syndrome; Glaucoma, Normal Tension, Susceptibility To; Mitochondrial Dna Depletion Syndrome 14 (cardioencephalomyopathic Type); Optic Atrophy 1; Optic Atrophy With Or Without Deafness, Ophthalmoplegia, Myopathy, Ataxia, And Neuropathy

Reference #: O60313 (UniProtKB)

Alt. Names/Synonyms: BERHS; Dynamin-like 120 kDa protein, form S1; Dynamin-like 120 kDa protein, mitochondrial; dynamin-like guanosine triphosphatase; FLJ12460; KIAA0567; largeG; MGM1; mitochondrial dynamin-like GTPase; MTDPS14; NPG; NTG; OPA1; OPA1 mitochondrial dynamin like GTPase; optic atrophy 1 (autosomal dominant); Optic atrophy protein 1

Gene Symbols: OPA1

Molecular weight: 111,631 Da

Basal Isoelectric point: 7.87 Predict pI for various phosphorylation states

Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology^®

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OPA1

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