OPA1 Antibody, FITC conjugated (PACO62157)
- SKU:
- PACO62157
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Description
抗体名: | OPA1 Antibody, FITC conjugated (PACO62157) |
抗体コード: | PACO62157 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Dynamin-like 120 kDa protein, mitochondrial protein (183-351AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | 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. May also play a role in mitochondrial genome maintenance. |
シノニム: | Dynamin-like 120 kDa protein, mitochondrial (EC 3.6.5.5) (Optic atrophy protein 1) [Cleaved into: Dynamin-like 120 kDa protein, form S1], OPA1, KIAA0567 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | 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 |
NCBI Summary: | 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] |
UniProt Code: | O60313 |
NCBI GenInfo Identifier: | 215274226 |
NCBI Gene ID: | 4976 |
NCBI Accession: | O60313.3 |
UniProt Secondary Accession: | O60313,D3DNW4, |
UniProt Related Accession: | O60313 |
Molecular Weight: | 112kDa |
NCBI Full Name: | Dynamin-like 120 kDa protein, mitochondrial |
NCBI Synonym Full Names: | OPA1, mitochondrial dynamin like GTPase |
NCBI Official Symbol: | OPA1 |
NCBI Official Synonym Symbols: | NPG; NTG; MGM1; BERHS; largeG; MTDPS14 |
NCBI Protein Information: | dynamin-like 120 kDa protein, mitochondrial |
UniProt Protein Name: | Dynamin-like 120 kDa protein, mitochondrial |
UniProt Synonym Protein Names: | Optic atrophy protein 1 |
Protein Family: | Dynamin-like 120 kDa protein |
UniProt Gene Name: | OPA1 |