Recombinant Human Ataxin 3 protein (ab116441)

製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号Q4VBR4
    • 生物種Human
    • 配列MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLELILFNSPEYQ RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ QQQQQQQQQQ QQQQGDLSGQ SSHPCERPAT SSGALGSDLG DAMSEEDMLQ AAVTMSLETV RNDLKTEGKK
    • 分子量42 kDa
    • 領域1 to 370

特性

Our Abpromise guarantee covers the use of ab116441 in the following tested applications.

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

  • アプリケーション

    SDS-PAGE

  • 精製度> 90 % SDS-PAGE.
    ab116441 was purified by proprietary chromatographic techniques and filter sterilized.
  • 製品の状態Liquid
  • 備考Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Please see notes section.

    pH: 7.50
    Constituents: 0.32% Tris HCl, 0.03% DTT, 0.29% Sodium chloride, 10% Glycerol

関連情報

  • 別名
    • AT3
    • Ataxin 3
    • ataxin 3 variant h
    • ataxin 3 variant m
    • ataxin 3 variant ref
    • Ataxin-3
    • ATX3
    • ATX3_HUMAN
    • ATXN3
    • EC 3.4.22.
    • JOS
    • Josephin
    • Machado Joseph disease
    • Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3)
    • Machado Joseph disease protein 1
    • Machado-Joseph disease protein 1
    • Machado-Joseph disease protein 1 homolog
    • MJD
    • MJD gene
    • MJD1
    • Olivopontocerebellar ataxia 3
    • OTTHUMP00000221583
    • OTTHUMP00000221585
    • OTTHUMP00000221586
    • OTTHUMP00000221587
    • OTTHUMP00000231995
    • OTTHUMP00000231997
    • Rsca3
    • SCA3
    • SCA3 gene
    • Spinocerebellar ataxia type 3 protein
    see all
  • 機能Interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription. Acts as a histone-binding protein that regulates transcription. Acts as a deubiquitinating enzyme.
  • 組織特異性Ubiquitous.
  • 関連疾患Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) [MIM:109150]; 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.
  • 配列類似性Contains 1 Josephin domain.
    Contains 3 UIM (ubiquitin-interacting motif) repeats.
  • 細胞内局在Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
  • Information by UniProt

Recombinant Human Ataxin 3 protein (ab116441) 使用論文

ab116441 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab116441.
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"