Overview

  • Product name
  • Description
    Rabbit polyclonal to QDPR
  • Tested applications
    Suitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Recombinant fragment containing a sequence corresponding to a region within amino acids 1-221 of Human QDPR. (Uniprot:P09417)

  • Positive control
    • Jurkat whole cell lysate, Mouse liver whole cell lysate and Human colon cancer tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab126150 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
WB 1/500 - 1/3000. Predicted molecular weight: 26 kDa.
IHC-P 1/100 - 1/500. Perform antigen retrieval using heat mediated 10mM Citrate buffer(pH6.0) or Tris-EDTA buffer (pH8.0)

Target

  • Function
    The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases.
  • Involvement in disease
    Defects in QDPR are the cause of BH4-deficient hyperphenylalaninemia type C (HPABH4C) [MIM:261630]; also called dihydropteridine reductase deficiency (DHPR deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to DHPR deficiency or quinoid dihydropteridine reductase deficiency (QDPR deficiency). HPABH4C is a rare autosomal recessive disorder characterized by hyperphenylalaninemia and severe neurologic symptoms (malignant hyperphenylalaninemia) including axial hypotonia and truncal hypertonia, abnormal thermogenesis, and microcephaly. These signs are attributable to depletion of the neurotransmitters dopamine and serotonin, whose syntheses are controlled by tryptophan and tyrosine hydroxylases that use BH-4 as cofactor. These patients do not respond to phenylalanine-restricted diet. HPABH4C is lethal if untreated.
  • Sequence similarities
    Belongs to the short-chain dehydrogenases/reductases (SDR) family.
  • Information by UniProt
  • Database links
  • Alternative names
    • 6,7 dihydropteridine reductase antibody
    • DHPR antibody
    • DHPR_HUMAN antibody
    • Dihydropteridine reductase antibody
    • HDHPR antibody
    • HPR antibody
    • PKU2 antibody
    • Qdpr antibody
    • Quinoid dihydropteridine reductase antibody
    • SDR33C1 antibody
    • Short chain dehydrogenase/reductase family 33C, member 1 antibody
    see all

Images

  • Anti-QDPR antibody (ab126150) at 1/1000 dilution + Mouse liver whole cell lysate at 50 µg

    Predicted band size : 26 kDa
  • Anti-QDPR antibody (ab126150) at 1/1000 dilution + Jurkat whole cell lysate at 30 µg

    Predicted band size : 26 kDa
  • ab126150, at 1/500 dilution, staining QDPR in Formalin-fixed, Paraffin-embedded Human Colon cancer tissue by Immunohistochemistry.

References

ab126150 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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