Recombinant人Ataxin 3 protein (ab86706)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • 种属Human
    • 序列MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ 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

技术指标

Our Abpromise guarantee covers the use of ab86706 in the following tested applications.

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

  • 应用

    SDS-PAGE

  • 纯度> 90 % SDS-PAGE.
    ab86706 is purified using conventional chromatography techniques. Endotoxin Level: < 1.0 EU per 1 µg of protein (determined by LAL method).
  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 10% Glycerol, 50mM Sodium chloride, 20mM Tris HCl, 2mM DTT, pH 7.5

常规信息

  • 别名
    • 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
  • 功能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.
  • 组织特异性Ubiquitous.
  • 疾病相关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.
  • 序列相似性Contains 1 Josephin domain.
    Contains 3 UIM (ubiquitin-interacting motif) repeats.
  • 细胞定位Nucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
  • Information by UniProt

Recombinant Human Ataxin 3 protein 图像

  • 15% SDS-PAGE showing ab86706 at approximately 42.4kDa (3µg).

Recombinant Human Ataxin 3 protein (ab86706)参考文献

ab86706 has not yet been referenced specifically in any publications.

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