Anti-COX1 antibody
-
概述
- 产品描述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); 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); 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). 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. Myoglobinuria consists of excretion of myoglobin in the urine. 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. Belongs to the heme-copper respiratory oxidase family.
- 产品名称Anti-COX1 antibody
- 分子量56 kDa
- 种属反应性Human,Mouse
- 验证应用WB
- 抗体类型兔多抗
- 免疫原Synthesized peptide derived from human protein . at AA range: 380-460
- 偶联Non-conjugated
-
性能
- 形态Liquid
- 浓度1 mg/mL.
- 存放说明Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
- 存储缓冲液Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
- 亚型IgG
- 纯化方式Immunogen affinity purified.
-
特别提示：本公司的所有产品仅可用于科研实验，严禁用于临床医疗及其他非科研用途！
