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細胞色素B抗體
  • 產品貨號:
    BN40984R
  • 中文名稱:
    細胞色素B抗體
  • 英文名稱:
    Rabbit anti-Cytochrome B Polyclonal antibody
  • 品牌:
    Biorigin
  • 貨號

    產品規格

    售價

    備注

  • BN40984R-100ul

    100ul

    ¥2360.00

    交叉反應:Human(predicted:Mouse,Rat,Pig) 推薦應用:WB,IHC-P,IHC-F,IF,Flow-Cyt,ELISA

  • BN40984R-200ul

    200ul

    ¥3490.00

    交叉反應:Human(predicted:Mouse,Rat,Pig) 推薦應用:WB,IHC-P,IHC-F,IF,Flow-Cyt,ELISA

產品描述

英文名稱Cytochrome B
中文名稱細胞色素B抗體
別    名MT-CYB; COB; Complex III subunit 3; Complex III subunit III; CYTB; Cytochrome b c1 complex subunit 3; Mitochondrially encoded cytochrome b; MTCYB; Ubiquinol cytochrome c reductase complex cytochrome b subunit; CYB_HUMAN.  
研究領域腫瘤  細胞生物  免疫學  信號轉導  轉錄調節因子  線粒體  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應Human,  (predicted: Mouse, Rat, Pig, )
產品應用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1ug/test IF=1:100-500 (石蠟切片需做抗原修復)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量43kDa
細胞定位細胞核 細胞漿 線粒體
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Cytochrome B:101-200/388 
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產品介紹Cytochrome b belongs to the cytochrome b family. It is a 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.

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 (By similarity).

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).

Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.

DISEASE:
Note=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).
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.
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.

Similarity:
Belongs to the cytochrome b family.

SWISS:
P00156

Gene ID:
4519

Database links:

Entrez Gene: 4519 Human

Omim: 516020 Human

SwissProt: P00156 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.




















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