Overview

  • Product name

  • Description

    Rabbit polyclonal to PQBP1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Cow, Gorilla, Orangutan
  • Immunogen

    Recombinant full length protein corresponding to Human PQBP1 aa 2-265.
    Database link: O60828

  • Positive control

    • IHC-P: Human testis tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab234790 in the following tested applications.

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

Application Abreviews Notes
IHC-P 1/20 - 1/200.

Target

  • 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
  • Database links

  • Alternative names

    • 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

Images

  • Paraffin-embedded human testis tissue stained for PQBP1 using ab234790 at 1/100 dilution in immunohistochemical analysis.

References

ab234790 has not yet been referenced specifically in any publications.

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