Product Description

OPA1 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES11922-50, ES11922-100

Lead times: approximately 7-10 business days, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: 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],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from part region of human protein

Isotype: IgG

Target: OPA1,

Specificity: OPA1 Polyclonal Antibody detects endogenous levels of protein.

Dilution: WB 1:500-2000 ELISA 1:5000-20000

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formulation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Expression: 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.

Observed Band (KD): 105kD

Fields: Spinocerebellar ataxia

Function: Disease: Defects in OPA1 are a cause of optic atrophy type 1 (OPA1) [MIM:165500]. OPA1 is a dominantly inherited optic neuropathy occurring in 1 in 50, 000 individuals that features progressive loss in visual acuity leading, in many cases, to legal blindness. Disease: Defects in OPA1 are the cause of optic atrophy 1 and deafness [MIM:125250]. Some individuals with mutations in OPA1 manifest also ophthalmoplegia and myopathy. 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. PTM: PARL-dependent proteolytic processing releases an antiapoptotic soluble form not required f

Subcellular Location: Mitochondrion inner membrane, Single-pass membrane protein Mitochondrion intermembrane space Mitochondrion membrane Detected at contact sites between endoplasmic reticulum and mitochondrion membranes.

Storage and Stability: -20°C/1 year

Gene Name: OPA1, KIAA0567

Protein Name: Dynamin-like 120 kDa protein, mitochondrial, Optic atrophy protein 1 [Cleaved into: Dynamin-like 120 kDa protein, form S1]

Human Gene ID: 4976

Human SwissProt Number: O60313

Mouse SwissProt Number: P58281

Rat SwissProt Number: Q2TA68

Research Use Only