Anti -OPA1 (Dynamin-like 120kD Protein, Mitochondrial, Optic Atrophy Protein 1, KIAA0567)

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

Chromosome: 3; NC_000003.11 (193310933..193415600). Location: 3q28-q29

Polyclonal

Rabbit

Recognizes human OPA1. Species Crossreactivity

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in 0.1M Tris-glycine, pH 7.4, 150mM sodium chloride, 0.05% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

OPA1 (optic atrophy 1) is a dynamin-related GTPase that plays a role in genome preservation and mitochondrial morphology. Localization of OPA1 is thought to be anchored to the inner membrane of the mitochondria. OPA1 is highly expressed in inner and outer retinal neurons and is important for normal mitochondrial function in those cells. Mutations in OPA1 are the major cause of autosomal dominant optic atrophy. Recent research has found that OPA1 also has the ability to protect neurons from excitotoxic injury and could be a potential clinical target to increase endurance of neurons post brain damage.

Antibodies; Abs to Proteins

Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in Western Blot and Immunohistochemistry.
Dilution: Western Blot: 0.1ug/ml
Immunohistochemistry: 1:1000

111,631 Da[Similar Products]

optic atrophy 1 (autosomal dominant)

dynamin-like 120 kDa protein, mitochondrial; optic atrophy protein 1; mitochondrial dynamin-like GTPase; dynamin-like guanosine triphosphatase

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; Membrane protein, integral; EC 3.6.5.5; Apoptosis; Mitochondrial

Chromosomal Location of Human Ortholog: 3q29

Cellular Component: nucleoplasm; mitochondrial outer membrane; mitochondrial crista; membrane; mitochondrion; dendrite; mitochondrial inner membrane; cytoplasm; 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; mitochondrial fission; mitochondrion organization and biogenesis; 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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