小脑脊髓共济失调蛋白3抗体

英文名称  Anti-ATXN3L 

中文名称  小脑脊髓共济失调蛋白3抗体 

别    名  ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg   

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human  

产品类型  一抗    

研究领域  细胞生物 免疫学 染色质和核信号 表观遗传学 泛素  

小脑脊髓共济失调蛋白3抗体蛋白分子量  predicted molecular weight: 41kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human ATXN3L 

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  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:50-200 

（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. 

小脑脊髓共济失调蛋白3抗体产品介绍 Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). 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. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

Function : Deubiquitinating enzyme that cleaves both 'Lys-48'-linked and 'Lys-63'-linked poly-ubiquitin chains (in vitro). 

Subcellular Location : Nucleus (By similarity). 

Similarity : Contains 1 Josephin domain.

Contains 2 UIM (ubiquitin-interacting motif) repeats. 

Database links : UniProtKB/Swiss-Prot: Q9H3M9.2