Anti-Huntingtin抗体[1A771] (ab13583)

概述

  • 产品名称Anti-Huntingtin抗体[1A771]
    参阅全部 Huntingtin 一抗
  • 描述
    小鼠单克隆抗体[1A771] to Huntingtin
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Human
  • 免疫原

    The antibody was raised against a fusion protein containing 62 glutamine amino acid repeats.

  • 阳性对照
    • Lymphoblast cell line extracts.
  • 常规说明

    Clone 1A771 binds to the expanded polyQ repeat form of Htt in WB, showing no detectable binding to normal Htt in WB.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: 0.02% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 纯度Protein G purified
  • 克隆单克隆
  • 克隆编号1A771
  • 同种型IgG1
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab13583 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 377 kDa.

靶标

  • 功能May play a role in microtubule-mediated transport or vesicle function.
  • 组织特异性Expressed in the brain cortex (at protein level). Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.
  • 疾病相关Defects in HTT are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum).
  • 序列相似性Belongs to the huntingtin family.
    Contains 10 HEAT repeats.
  • 结构域The N-terminal Gln-rich and Pro-rich domain has great conformational flexibility and is likely to exist in a fluctuating equilibrium of alpha-helical, random coil, and extended conformations.
  • 翻译后修饰Cleaved by apopain downstream of the polyglutamine stretch. The resulting N-terminal fragment is cytotoxic and provokes apoptosis.
    Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.
  • 细胞定位Cytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AI256365 antibody
    • C430023I11Rik antibody
    • HD antibody
    • HD protein antibody
    • HD_HUMAN antibody
    • HDH antibody
    • HTT antibody
    • Huntingtin antibody
    • HUNTINGTON CHOREA antibody
    • Huntington disease protein antibody
    • Huntington's disease protein homolog antibody
    • IT 15 antibody
    • IT15 antibody
    • OTTMUSP00000026909 antibody
    • ZHD antibody
    see all

Anti-Huntingtin antibody [1A771] 图像



  • Predicted band size : 377 kDa

Anti-Huntingtin antibody [1A771] (ab13583)参考文献

ab13583 has not yet been referenced specifically in any publications.

Product Wall

Thank you for getting back to me with further details. Once again I am sorry to hear of the difficulties that you have been experiencing with this antibody by western blotting. I am certainly prepared to offer you a credit note to the value of one ...

Read More

Thank you for your enquiry. I am sorry to hear that you have been having difficulties with this antibody. Quality is important to Abcam. You are using an ideal system for the testing of this antibody and have tried many of the suggestions that ...

Read More

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"