Overview

  • Product name
    Recombinant Human PCBD1 protein
  • Protein length
    Full length protein

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHH SSGLVPRGSH MAGKAHRLSA EERDQLLPNL RAVGWNELEG RDAIFKQFHF KDFNRAFGFM TRVALQAEKL DHHPEWFNVY NKVHITLSTH ECAGLSERDI NLASFIEQVA VSMT
    • Molecular weight
      14 kDa including tags
    • Amino acids
      1 to 104
    • Tags
      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab100942 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.
    ab100942 was purified using conventional chromatography techniques.
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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, 20mM Tris HCl, 1mM DTT, pH 8

General Info

  • Alternative names
    • 4 alpha hydroxy tetrahydropterin dehydratase
    • 4-alpha-hydroxy-tetrahydropterin dehydratase
    • 6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha
    • 6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1)
    • DCoH
    • Dimerization cofactor of hepatic nuclear factor 1 alpha
    • Dimerization cofactor of hepatocyte nuclear factor 1 alpha
    • Dimerization cofactor of hepatocyte nuclear factor 1-alpha
    • Dimerization cofactor of HNF1
    • Dimerizing cofactor for HNF1
    • PCBD
    • PCBD 1
    • PCBD1
    • PCD
    • Phenylalanine hydroxylase stimulating protein
    • Phenylalanine hydroxylase-stimulating protein
    • PHS
    • PHS_HUMAN
    • Pterin 4 alpha carbinolamine dehydratase
    • Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha
    • Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1)
    • Pterin carbinolamine dehydratase
    • Pterin-4-alpha-carbinolamine dehydratase
    see all
  • Function
    Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription. Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity.
  • Involvement in disease
    Defects in PCBD1 are the cause of BH4-deficient hyperphenylalaninemia type D (HPABH4D) [MIM:264070]; also known as hyperphenylalaninemia with primapterinuria. HPABH4D is characterized by the excretion of 7-substituted pterins in the urine of affected patients.
  • Sequence similarities
    Belongs to the pterin-4-alpha-carbinolamine dehydratase family.
  • Cellular localization
    Cytoplasm. Nucleus. Cytoplasmic and/or nuclear.
  • Information by UniProt

Images

  • ab100942, at 3 µg, on 15% SDS PAGE.

References

ab100942 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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