产品详情
  • 产品名称:Anti-ATXN3L抗体

  • 产品型号:小脑脊髓共济失调蛋白3抗体
  • 产品厂商:科研抗体
  • 产品价格:0
  • 折扣价格:0
  • 产品文档:
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简单介绍:
Anti-ATXN3L抗体供应商为您提供抗体的售前、售中、售后服务,现货供应,保证实验结果,无效可以免费退换,广泛应用于IHC、WB、 IF、IP、ELISA,质量保证,另外本公司生产经营各种单克隆抗体、多克隆抗体,标记二抗、**印迹常用试剂、**组化常用试剂,Anti-ATXN3L抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍:
Rabbit  Anti-ATXN3L
Cat. Number:
YB4807R
Quantity size:
0.1ml
Concentration:
Anti-ATXN3L抗体1mg/ml   Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
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. Anti-ATXN3L抗体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.
Also known as:
Anti-ATXN3L抗体ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.
Specificity:
Rabbit Polyclonal IgG, affinity purified by Protein A.
Reacts with: Human, .
Immunogen: Anti-ATXN3L抗体KLH conjugated synthetic peptide derived from human ATXN3L.
Predicted Molecular Weight: 41kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
Anti-ATXN3L抗体WB=1:100-500  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:50-200   
Not yet tested in other applications. 
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