Cytochrome b anticorps (AA 331-380)

N° du produit ABIN8122978

Cet anticorps anti-Cytochrome b Polyclonal Lapin (ABIN8122978) détecte spécifiquement Cytochrome b dans WB et ELISA. L’anticorps est réactif avec des échantillons de Humain et Souris.

Aperçu rapide pour Cytochrome b anticorps (AA 331-380) (ABIN8122978)

Antigène: Cytochrome b (MT-CYB) (Mitochondrially Encoded Cytochrome B (MT-CYB))

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Cytochrome b est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-Cytochrome b anticorps

Épitope: AA 331-380

Fonction: Cytochrome b Antibody

Specificité: Cytochrome b Polyclonal Antibody detects endogenous levels of Cytochrome b

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

Immunogène: Synthesized peptide derived from human Cytochrome b. at AA range: 331-380

Isotype: IgG

Information d'application

Indications d'application: WB 1:500-2000, ELISA 1:10000-20000

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Liquid in PBS containing 50 % glycerol, 0.5 % BSA 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 Cytochrome b

Antigène: Cytochrome b (MT-CYB) (Mitochondrially Encoded Cytochrome B (MT-CYB))

Autre désignation: Cytochrome b

Sujet:

Synonyms: Cytochrome b (Complex III subunit 3) (Complex III subunit III) (Cytochrome b-c1 complex subunit 3) (Ubiquinol-cytochrome-c reductase complex cytochrome b subunit)

Background: cofactor:Binds 2 heme groups non-covalently.,disease:Defects in MT-CYB are a rare cause of mitochondrial dysfunction underlying different myopathies. They include mitochondrial encephalomyopathy, hypertrophic cardiomyopathy (HCM), and sporadic mitochondrial myopathy (MM). In mitochondrial myopathy, exercise intolerance is the predominant symptom. Additional features include lactic acidosis, muscle weakness and/or myoglobinuria. Defects in MTCYB are also found in cases of exercise intolerance accompanied by deafness, mental retardation, retinitis pigmentosa, cataract, growth retardation, epilepsy (multisystem disorder).,disease:Defects in MT-CYB are the cause of cardiomyopathy infantile histiocytoid (CMIH) [MIM:500000]. CMIH is characterized by the presence of pale granular foamy histiocyte-like cells within the myocardium. It usually affects children younger than 2 years of age, with a clear predominance of females over males. Infants present with dysrhythmia or cardiac arrest, and the clinical course is usually fulminant, sometimes simulating sudden infant death syndrome.,disease:Defects in MT-CYB contribute to 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.,function:Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis.,miscellaneous:Heme 1 (or BL or b562) is low-potential and absorbs at about 562 nm, and heme 2 (or BH or b566) is high-potential and absorbs at about 566 nm.,similarity:Belongs to the cytochrome b family.,subunit:The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).,

Gene Name: MT-CYB

Protein Name: Cytochrome b

ID gène: 4519

UniProt: P00156

Pathways: Proton Transport