Cytochrome b Antikörper (AA 331-380)

Produktnummer ABIN8122978

Der Kaninchen Polyklonal anti-Cytochrome b Antikörper (ABIN8122978) detektiert spezifisch Cytochrome b in WB und ELISA. Dieser Antikörper reagiert spezifisch mit Proben aus Human und Maus.

Kurzübersicht für Cytochrome b Antikörper (AA 331-380) (ABIN8122978)

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

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Cytochrome b Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-Cytochrome b Antikörper

Bindungsspezifität: AA 331-380

Verwendungszweck: Cytochrome b Antibody

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

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

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:500-2000, ELISA 1:10000-20000

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Liquid in PBS containing 50 % glycerol, 0.5 % BSA and 0.02 % sodium azide.

Konservierungsmittel: Sodium azide

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C/1 year

Haltbarkeit: 12 months

Details zu Cytochrome b

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

Andere Bezeichnung: Cytochrome b

Hintergrund:

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

Gen-ID: 4519

UniProt: P00156

Pathways: Proton Transport