概述

  • 产品名称Anti-PQBP1抗体
    参阅全部 PQBP1 一抗
  • 描述
    小鼠单克隆抗体to PQBP1
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Recombinant Fragment
    预测可用于: Human
  • 免疫原

    Recombinant fragment: LAPYPKSKKA VSRKDEELDP MDPSSYSDAP RGTWSTGLPK RNEAKTGADT, corresponding to amino acids 184-265 of human PQBP1 (NP_005701) with a 26 kDa tag.

  • 阳性对照
    • Immunogen

性能

应用

Our Abpromise guarantee covers the use of ab77041 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
ELISA
  • 应用说明ELISA: Use at an assay dependent dilution.

    WB: 1/500 - 1/1000. Predicted molecular weight: 30 kDa.
    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.

  • 靶标

    • 功能May suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. Can activate transcription directly or via association with the transcription machinery. May be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit.
    • 组织特异性Widely expressed with high level in heart, skeletal muscle, pancreas, spleen, thymus, prostate, ovary, small intestine and peripheral blood leukocytes.
    • 疾病相关Defects in PQBP1 are the cause of Renpenning syndrome 1 (RENS1) [MIM:309500]; also known as Sutherland-Haan X-linked mental retardation syndrome (SHS) or X-linked mental retardation syndromes MRXS3/MRXS8/MRX55. The clinical features are mental retardation, microcephaly, short stature, and small testes. The craniofacies tends to be narrow and tall with upslanting palpebral fissures, abnormal nasal configuration, cupped ears, and short philtrum. The nose may appear long or bulbous, with overhanging columella. Less consistent manifestations include ocular colobomas, cardiac malformations, cleft palate, and anal anomalies. RENS1 is more frequently in males than in females where little or no expression is found.
    • 序列相似性Contains 1 WW domain.
    • 结构域The WW domain may play a role as a transcriptional activator directly or via association with the transcription machinery. The WW domain mediates interaction with C-terminal domain of RNA polymerase II large subunit.
    • 细胞定位Nucleus. Co-localized with POU3F2. Co-localized with ATXN1 in nuclear inclusion bodies.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 38 kDa nuclear protein containing a WW domain antibody
      • Mental retardation, X linked 55 antibody
      • MRX55 antibody
      • MRXS3 antibody
      • MRXS8 antibody
      • Npw38 antibody
      • Nuclear protein containing WW domain 38 kD antibody
      • OTTHUMP00000025808 antibody
      • Polyglutamine binding protein 1 antibody
      • Polyglutamine tract binding protein 1 antibody
      • Polyglutamine tract-binding protein 1 antibody
      • Polyglutamine-binding protein 1 antibody
      • PQBP 1 antibody
      • PQBP-1 antibody
      • PQBP1 antibody
      • PQBP1_HUMAN antibody
      • RENS1 antibody
      • SHS antibody
      • Sutherland Haan X linked mental retardation syndrome antibody
      see all

    Anti-PQBP1 antibody 图像

    • Anti-PQBP1 antibody (ab77041) at 1/500 dilution + immunogen at 0.2 µg

      Secondary
      Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/5000 dilution

      Predicted band size : 30 kDa
      Observed band size : 35 kDa (why is the actual band size different from the predicted?)

    Anti-PQBP1 antibody (ab77041)参考文献

    ab77041 has not yet been referenced specifically in any publications.

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