Anti -Opa1 (Optic Atrophy 1, Optic Atrophy 1 Gene Protein, Optic Atrophy Protein 1, Dynamin-like 120kD Protein)

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

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

Polyclonal

Rabbit

Species Crossreactivity: This antibody reacts with the human protein. It has not been tested against any other species.

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in Tris-glycine, 150mM sodium chloride, pH 7.2.

May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot Store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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.

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. Eight transcript variants encoding different isoforms, resulting from alternative splicing of exon 4 and two novel exons named 4b and 5b, have been reported for this gene.

Antibodies; Abs to Proteins

Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in Western Blot and Immunohistochemistry.

33,060 Da[Similar Products]

optic atrophy 1 (autosomal dominant)

dynamin-like 120 kDa protein, mitochondrial; OTTHUMP00000195064; OTTHUMP00000195065; optic atrophy protein 1; mitochondrial dynamin-like GTPase; mitochondrial dynamin-like 120 kDa protein

OPA1 protein

Q6PEK6_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]

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

Chromosomal Location of Human Ortholog: 3q29

Cellular Component: nucleoplasm; mitochondrial outer membrane; mitochondrial crista; membrane; mitochondrion; mitochondrial inner membrane; dendrite; 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; 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

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.