Overview

  • Product name

  • Description

    Rabbit polyclonal to QDPR/DHPR
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IF, IHC-P, WBmore details
  • Species reactivity

    Reacts with: Rat, Human, Pig
  • Immunogen

    Recombinant full length protein (His-tag) corresponding to Human QDPR/DHPR aa 1-244. (Expressed in E.coli).
    Sequence:

    MAAAAAAGEARRVLVYGGRGALGSRCVQAFRARNWWVASVDVVENEEASA SIIVKMTDSFTEQADQVTAEVGKLLGEEKVDAILCVAGGWAGGNAKSKSL FKNCDLMWKQSIWTSTISSHLATKHLKEGGLLTLAGAKAALDGTPGMIGY GMAKGAVHQLCQSLAGKNSGMPPGAAAIAVLPVTLDTPMNRKSMPEADFS SWTPLEFLVETFHDWITGKNRPSSGSLIQVVTTEGRTELTPAYF


    Database link: P09417

  • Positive control

    • IHC-P: Human cerebrum tissue. ICC/IF: HepG2 cells. WB: Recombinant human QDPR/DHPR protein; HepG2 cell lysate; Pig liver and brain lysate; Rat liver lysate.
  • General notes

    Previously labelled as QDPR. 

Properties

  • Form

    Liquid
  • Storage instructions

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

    pH: 7.4
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    Antigen-specific affinity chromatography followed by Protein A affinity chromatography.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab232983 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
ICC/IF 1/5 - 1/20.
IHC-P Use a concentration of 5 - 20 µg/ml.
WB Use a concentration of 0.5 - 2 µg/ml. Predicted molecular weight: 26 kDa.

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

  • HepG2 (human liver hepatocellular carcinoma cell line) cells labelling QDPR/DHPR (green) using ab232983 at 20 ug/ml in ICC/IF. FITC staining.

  • All lanes : Anti-QDPR/DHPR antibody (ab232983) at 2 µg/ml

    Lane 1 : HepG2 (human liver hepatocellular carcinoma cell line) cell lysate.
    Lane 2 : Pig liver tissue lysate
    Lane 3 : Pig brain tissue lysate.
    Lane 4 : Rat brain tissue lysate.

    Secondary
    All lanes : HRP-Linked Guinea pig Anti-Rabbit Ab at 1/2000 dilution

    Predicted band size: 26 kDa

  • Anti-QDPR/DHPR antibody (ab232983) at 2 µg/ml + Recombinant human QDPR/DHPR protein.

    Predicted band size: 26 kDa

  • Formalin-fixed, paraffin-embedded human cerebrum tissue stained for QDPR/DHPR using ab232983 at 20ug/ml in immunohistochemical analysis. DAB staining.

References

ab232983 has not yet been referenced specifically in any publications.

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