COX1 anticorps (AA 380-460)

N° du produit ABIN8120859

L’anticorps anti-COX1 Polyclonal Lapin est utilisé pour la détection de COX1 dans des échantillons de Humain et Souris. Il a été validé pour WB et ELISA.

Aperçu rapide pour COX1 anticorps (AA 380-460) (ABIN8120859)

Antigène: COX1 (Cytochrome C Oxidase Subunit I (COX1))

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp COX1 est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-COX1 anticorps

Épitope: AA 380-460

Fonction: COX1 Antibody

Specificité: COX1 Polyclonal Antibody detects endogenous levels of protein.

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Immunogène: Synthesized peptide derived from human protein . at AA range: 380-460

Isotype: IgG

Information d'application

Indications d'application: WB 1:500-2000 ELISA 1:5000-20000

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Liquid in PBS containing 50 % glycerol, and 0.02 % sodium azide.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C/1 year

Date de péremption: 12 months

Détails sur COX1

Antigène: COX1 (Cytochrome C Oxidase Subunit I (COX1))

Autre désignation: COX1

Sujet:

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)

ID gène: 4512

UniProt: P00395

Pathways: Proton Transport