Recombinant人Huntingtin protein (ab112300)

概述

  • 产品名称
    Recombinant人Huntingtin protein
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      AVAEEPLHRPKKELSATKKDRVNHCLTICENIVAQSVRNSPEFQKLLGIA MELFLLCSDDAESDVRMVADECLNKVIKALMDSNLPRLQLELYKEIKKNG APRSLRAALW
    • 分子量
      38 kDa including tags
    • 氨基酸
      81 to 190
    • 标签
      GST tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab112300 in the following tested applications.

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

  • 生物活性
    useful for Antibody Production and Protein Array
  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式
    Liquid
  • 补充说明
    useful for Antibody Production and Protein Array
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.79% Tris HCl, 0.31% Glutathione
    Note: Glutathione is reduced

常规信息

  • 别名
    • AI256365
    • C430023I11Rik
    • HD
    • HD protein
    • HD_HUMAN
    • HDH
    • HTT
    • Huntingtin
    • HUNTINGTON CHOREA
    • Huntington disease protein
    • Huntington's disease protein homolog
    • IT 15
    • IT15
    • OTTMUSP00000026909
    • ZHD
    see all
  • 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

Recombinant Human Huntingtin protein 图像

  • ab112300 analysed on a 12.5% SDS-PAGE stained with Coomassie Blue.

Recombinant Human Huntingtin protein (ab112300)参考文献

ab112300 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab112300.
Please use the links above to contact us or submit feedback about this product.

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

注册