COX1 rabbit pAb
Product Sizes
50UL
£ POA
ES9123-50UL
100UL
£ POA
ES9123-100UL
About this Product
- SKU:
- ES9123
- Application:
- Western Blot, ELISA
- Clonality:
- Polyclonal
- Concentration:
- 1 mg/ml
- Extra Details:
- 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.;
- Immunogen:
- Synthesized peptide derived from human protein . at AA range: 380-460
- Isotype:
- IgG
- Reactivities:
- Human, Mouse
- Shipping Conditions:
- Blue Ice
- Source:
- Rabbit
- Storage Conditions:
- -20[o]C
- Supplier:
- ELK Biotechnology
- Type:
- Antibodies: Polyclonal Antibody
