COX1 抗体 (AA 380-460)
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Quick Overview for COX1 抗体 (AA 380-460) (ABIN8120859)
抗原
See all COX1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 380-460
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原理
- COX1 Antibody
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特异性
- COX1 Polyclonal Antibody detects endogenous levels of protein.
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纯化方法
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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免疫原
- Synthesized peptide derived from human protein . at AA range: 380-460
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亚型
- IgG
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应用备注
- WB 1:500-2000 ELISA 1:5000-20000
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Liquid in PBS containing 50 % glycerol, and 0.02 % sodium azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C/1 year
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有效期
- 12 months
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- COX1 (Cytochrome C Oxidase Subunit I (COX1))
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别名
- COX1
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背景
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Background: catalytic activity:4 ferrocytochrome c + O(2) + 4 H(+) = 4 ferricytochrome c + 2 H(2)O.,disease:Defects in MT-CO1 are a cause of anemia sideroblastic acquired idiopathic (AISA) [MIM:516030], a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria.,disease:Defects in MT-CO1 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110], also called mitochondrial complex IV deficiency. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in MT-CO1 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,disease:Defects in MT-CO1 are associated with recurrent myoglobinuria [MIM:550500]. Myoglobinuria consists of excretion of myoglobin in the urine.,function:Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B.,pathway:Energy metabolism, oxidative phosphorylation.,similarity:Belongs to the heme-copper respiratory oxidase family.,
Gene Name: MT-CO1 COI COXI MTCO1
Protein Name: Cytochrome c oxidase subunit 1 (EC 1.9.3.1) (Cytochrome c oxidase polypeptide I)
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基因ID
- 4512
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UniProt
- P00395
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途径
- Proton Transport
抗原
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