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COQ8A Rabbit pAb (bs-8071R)  
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產品編號 bs-8071R
英文名稱 COQ8A Rabbit pAb
中文名稱 伴侶蛋白bc1同源復合體抗體
別    名 mitochondrial; aarF domain containing protein kinase 3; aarF domain-containing protein kinase 3; ADCK 3; ADCK3; ADCK3_HUMAN; CABC 1; Chaperone ABC1(activity of bc1 complex S.pombe) like; Chaperone ABC1 activity of bc1 complex homolog; Chaperone ABC1 like; Chaperone activity of bc1 complex like; Chaperone activity of bc1 complex like mitochondrial; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q8 homolog; COQ 8; COQ8.  
研究領域 腫瘤  細胞生物  神經生物學  信號轉導  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human,Rat (predicted: Mouse,Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 72 kDa
檢測分子量
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COQ8A: 501-647/647 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 This gene encodes a mitochondrial protein similar to yeast ABC1, which functions in an electron-transferring membrane protein complex in the respiratory chain. It is not related to the family of ABC transporter proteins. Expression of this gene is induced by the tumor suppressor p53 and in response to DNA damage, and inhibiting its expression partially suppresses p53-induced apoptosis. Alternatively spliced transcript variants have been found; however, their full-length nature has not been determined. [provided by RefSeq, Jul 2008]

Function:
May be a chaperone-like protein essential for the properconformation and functioning of protein complexes in therespiratory chain.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Ubiquitously expressed with a relativelygreater abundance in heart and skeletal muscle.

DISEASE:
Defects in ADCK3 are the cause of coenzyme Q10deficiency, primary, type 4 (COQ10D4) [MIM:612016]. An autosomalrecessive disorder characterized by childhood-onset of cerebellarataxia and exercise intolerance. Patient manifest gait ataxia andcerebellar atrophy with slow progression. Additional featuresinclude brisk tendon reflexes and Hoffmann sign, variablepsychomotor retardation and variable seizures.

Similarity:
Belongs to the protein kinase superfamily. ADCKprotein kinase family.
Contains 1 protein kinase domain.

SWISS:
Q8NI60

Gene ID:
56997

Database links:

Entrez Gene: 56997 Human

Entrez Gene: 67426 Mouse

Entrez Gene: 360887 Rat

Omim: 606980 Human

SwissProt: Q8NI60 Human

SwissProt: Q60936 Mouse

SwissProt: Q5BJQ0 Rat

Unigene: 118241 Human

Unigene: 38330 Mouse

Unigene: 160865 Rat



產品圖片
Sample: HepG2(Human) Cell Lysate at 30 ug Primary: Anti- CABC1 (bs-8071R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 72 kD Observed band size: 75 kD
Tissue/cell: rat heart tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-CABC1 Polyclonal Antibody, Unconjugated(bs-8071R) 1:500, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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