Overview

  • Product name
    Recombinant Human PQBP1 protein
  • Protein length
    Full length protein

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHH SSGLVPRGSH MGSHMPLPVA LQTRLAKRGI LKHLEPEPEE EIIAEDYDDD PVDYEATRLE GLPPSWYKVF DPSCGLPYYW NADTDLVSWL SPHDPNSVVT KSAKKLRSSN ADAEEKLDRS HDKSDRGHDK SDRSHEKLDR GHDKSDRGHD KSDRDRERGY DKVDRERERD RERDRDRGYD KADREEGKER RHHRREELAP YPKSKKAVSR KDEELDPMDP SSYSDAPRGT WSTGLPKRNE AKTGADTTAA GPLFQQRPYP SPGAVLRANA EASRTKQQD
    • Molecular weight
      33 kDa including tags
    • Amino acids
      1 to 265
    • Tags
      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab126687 in the following tested applications.

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

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • Mass spectrometry
    MALDI-TOF
  • Purity
    > 95 % SDS-PAGE.
    purified by using conventional chromatography techniques.
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 20% Glycerol, 0.58% Sodium chloride

General Info

  • Alternative names
    • 38 kDa nuclear protein containing a WW domain
    • Mental retardation, X linked 55
    • MRX55
    • MRXS3
    • MRXS8
    • Npw38
    • Nuclear protein containing WW domain 38 kD
    • OTTHUMP00000025808
    • Polyglutamine binding protein 1
    • Polyglutamine tract binding protein 1
    • Polyglutamine tract-binding protein 1
    • Polyglutamine-binding protein 1
    • PQBP 1
    • PQBP-1
    • PQBP1
    • PQBP1_HUMAN
    • RENS1
    • SHS
    • Sutherland Haan X linked mental retardation syndrome
    see all
  • Function
    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.
  • Tissue specificity
    Widely expressed with high level in heart, skeletal muscle, pancreas, spleen, thymus, prostate, ovary, small intestine and peripheral blood leukocytes.
  • Involvement in disease
    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.
  • Sequence similarities
    Contains 1 WW domain.
  • Domain
    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.
  • Cellular localization
    Nucleus. Co-localized with POU3F2. Co-localized with ATXN1 in nuclear inclusion bodies.
  • Information by UniProt

Images

  • 15% SDS-PAGE using 3µg of ab126687.

References

ab126687 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"

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