Recombinant Human Huntingtin protein (ab112300)

製品の概要

  • 製品名Recombinant Human Huntingtin protein
  • タンパク質長Protein fragment

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P42858
    • 生物種Human
    • 配列AVAEEPLHRPKKELSATKKDRVNHCLTICENIVAQSVRNSPEFQKLLGIA MELFLLCSDDAESDVRMVADECLNKVIKALMDSNLPRLQLELYKEIKKNG APRSLRAALW
    • 分子量38 kDa including tags
    • 領域81 to 190
    • タグGST tag N-Terminus

特性

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.

  • 生理活性useful for Antibody Production and Protein Array
  • アプリケーション

    ELISA

    SDS-PAGE

    Western blot

  • 製品の状態Liquid
  • 備考useful for Antibody Production and Protein Array
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能May play a role in microtubule-mediated transport or vesicle function.
  • 組織特異性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.
  • 関連疾患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).
  • 配列類似性Belongs to the huntingtin family.
    Contains 10 HEAT repeats.
  • ドメイン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.
  • 翻訳後修飾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.
  • 細胞内局在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 画像

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

Recombinant Human Huntingtin protein (ab112300) 使用論文

ab112300 has not yet been referenced specifically in any publications.

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