BERHS; MGM1; MTDPS14; NPG; NTG; largeG

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

Polyclonal

IgG

Rabbit

Affinity Purification

Store at -20 degree C. 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.

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.

Primary antibody

Western Blot (WB)

WB: 1:500 - 1:2000

Western blot analysis of extracts of various cell lines, using OPA1 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 10s.

112kDa

OPA1, mitochondrial dynamin like GTPase

dynamin-like 120 kDa protein, mitochondrial

Dynamin-like 120 kDa protein, mitochondrial

KIAA0567??[Similar Products]

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

Chromosomal Location of Human Ortholog: 3q29

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

Molecular Function: GTPase activity; microtubule binding; protein binding

Biological Process: axon transport of mitochondrion; inner mitochondrial membrane organization and biogenesis; mitochondrial fission; mitochondrial fusion; mitochondrial genome maintenance; mitochondrion organization and biogenesis; regulation of apoptosis; 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

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