產品貨號 :  mlR8071

英文名稱 :  CABC1

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

交叉反應 :   Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,

產品應用 :  WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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 :  72kDa

細胞定位 :  細胞漿 線粒體

性    狀 :  Lyophilized or Liquid

濃    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human ADCK3/CABC1:501-647/647

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 :  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

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

Important Note:

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

產品圖片