伴侣蛋白bc1同源复合体抗体

英文名称  Anti-ADCK3/CABC1 

中文名称  伴侣蛋白bc1同源复合体抗体 

别    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. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg    

抗体来源  Rabbit  

克隆类型  polyclonal 

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

产品类型  一抗    

研究领域  肿瘤 细胞生物 神经生物学 信号转导  

伴侣蛋白bc1同源复合体抗体蛋白分子量  predicted molecular weight: 72kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human ADCK3/CABC1 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  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. 

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

伴侣蛋白bc1同源复合体抗体产品介绍 May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.

Tissue specificity:Ubiquitously expressed with a relatively greater abundance in heart and skeletal muscle.

Involvement in disease:

Defects in ADCK3 are a cause of coenzyme Q10 deficiency (CoQ10 deficiency). CoQ10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy. Defects in ADCK3 are the cause of spinocerebellar ataxia autosomal recessive type 9 (SCAR9) [MIM:612016]; also known as autosomal recessive cerebellar ataxia type 2 (ARCA2). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR9 is an autosomal recessive form characterized by gait ataxia and cerebellar atrophy with slow progression and few associated features. Patients can manifest brisk tendon reflexes and Hoffmann sign, mild psychomotor retardation, mild axonal degeneration of the sural nerve, exercise intolerance and elevated serum lactate.

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.

Database links : UniProtKB/Swiss-Prot: Q8NI60.1