Optic Atrophy 1 (Autosomal Dominant) (OPA1) (AA 708-830) antibody

Antigen: Optic Atrophy 1 (Autosomal Dominant) (OPA1)

Synonyms: NPG, NTG, MGM1, largeG, FLJ12460, KIAA0567

Binding Site: AA 708-830

Clonality: Monoclonal (18)

Host: Mouse

Reactivity: Human

Conjugate: Un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF)

Catalog no.: ABIN968891

Additional Information

Alternative name: OPA1

Immunogen: Human OPA1

Cross-Reactivity: Dog (Canine), Rat (Rattus), Mouse (Murine), Chicken

Format: Liquid

Isotype: IgG1

Clone: 18

Description: Three major GTP-binding protein families include trimeric and low molecular weight G-proteins, as well as a family of large proteins homologous to dynamin. The dynamin family contains proteins with diverse structure and function, but highly homologous N-terminal GTPase domains. A subgroup of the dynamin G-protein-binding family includes the mitochondrial proteins Drp1/Dnm1, Mgm1, and OPA1. The latter protein is mutated in dominant optic atrophy, a disease that involves loss of visual acuity and atrophy of the optic nerve. OPA1 is expressed in heart, brain, liver, and kidney. The sequence of OPA1 includes an N-terminal region that contains a mitochondrial targeting domain and three GTP-binding motifs. The overexpression of OPA1 in Cos-7 cells shows co-localization with cytochrome c in mitochondria, and leads to alterations in mitochondrial morphology from a characteristic tubuluar shape to a vesicular pattern. Thus, OPA1 may have roles in mitochondrial biogenesis that are critical for normal cell function. This antibody is routinely tested by western blot analysis.

Characteristics: 1. Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2. Please refer to us for technical protocols.
3. Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
4. Source of all serum proteins is from USDA inspected abattoirs located in the United States.

Molecular Weight: 80-100 kDa

Comments:

Related Products: ABIN968586, ABIN967389

Application Details

Concentration: 250 µg/ml

Purity: Purified

Purification: Purified from tissue culture supernatant or ascites by affinity chromatography.

Buffer: Aqueous buffered solution containing BSA, glycerol.

Preservative: 0.09% Sodium azide.

Storage: Store undiluted at -20° C.

Research Area: Neurology

Restrictions: For Research Use only

Images

Western blot analysis of OPA1 on a K-562 cell lysate (Human bone marrow myelogenous leukemia, ATCC CCL-243). Lane 1: 1:500, lane 2: 1000, lane 3: 1: 2000 dilution of the mouse anti- OPA1 antibody.

Immunofluorescence staining of COS-7 cells (African Green Monkey SV40 transformed kidney cells, ATCC CRL-1651).

Publications

Product: Delettre, Lenaers, Griffoin et al.: "Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy." in: Nature genetics, Vol. 26, Issue 2, pp. 207-10, 2000 (PubMed).

Alexander, Votruba, Pesch et al.: "OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28." in: Nature genetics, Vol. 26, Issue 2, pp. 211-5, 2000 (PubMed).

Misaka, Miyashita, Kubo: "Primary structure of a dynamin-related mouse mitochondrial GTPase and its distribution in brain, subcellular localization, and effect on mitochondrial morphology." in: The Journal of biological chemistry, Vol. 277, Issue 18, pp. 15834-42, 2002 (PubMed).