OPA1, Polyclonal Antibody

KIAA0567; Dynamin-like 120 kDa protein, mitochondrial; Optic atrophy protein 1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Recognizes endogenous levels of OPA1 protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-OPA1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to OPA1

Western Blot (WB)

WB (1/500 - 1/1000)

Western blot analysis of OPA1 expression in A549 (A), Raw264.7 (B), PC12 (C) whole cell lysates.

optic atrophy 1 (autosomal dominant)

dynamin-like 120 kDa protein, mitochondrial

Dynamin-like 120 kDa protein, mitochondrial

KIAA0567  [Similar Products]

OPA1_HUMAN

This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Mutations in this gene have been associated with optic atrophy type 1, which is a dominantly inherited optic neuropathy resulting in progressive loss of visual acuity, leading in many cases to legal blindness. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]

OPA1: a dynamin-related GTPase required for mitochondrial fusion and regulation of apoptosis. May form a diffusion barrier for proteins stored in mitochondrial cristae. 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. Defects in OPA1 are the cause of optic atrophy 1 and deafness. Eight splice-variant isoforms have been described.

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

Chromosomal Location of Human Ortholog: 3q29

Cellular Component: nucleoplasm; mitochondrial crista; mitochondrial outer membrane; membrane; mitochondrion; dendrite; cytoplasm; mitochondrial inner membrane; integral to membrane; mitochondrial intermembrane space

Molecular Function: GTPase activity; protein binding; GTP binding; magnesium ion binding

Biological Process: inner mitochondrial membrane organization and biogenesis; mitochondrion organization and biogenesis; mitochondrial fission; mitochondrial genome maintenance; axon transport of mitochondrion; visual perception; mitochondrial fusion; apoptosis; metabolic process; neural tube closure

Disease: Glaucoma, Normal Tension, Susceptibility To; Optic Atrophy With Or Without Deafness, Ophthalmoplegia, Myopathy, Ataxia, And Neuropathy; Optic Atrophy 1

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