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QDPR recombinant protein

Scan QR to view Datasheet Catalog #    MBS844809
Testing Data
Unit / Price
0.1 mg  /  $365 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

QDPR, Recombinant Protein

 Also Known As   

QDPR, human recombinant

 Product Synonym Names    Quinoid dihydropteridine reductase; DHPR; FLJ42391; PKU2; SDR33C1
 Product Gene Name   

QDPR recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    MGSSHHHHHH SSGLVPRGSH MGSMAAAAAA GEARRVLVYG GRGALGSRCV QAFRARNWWV ASVDVVENEE ASASIIVKMT DSFTEQADQV TAEVGKLLGE EKVDAILCVA GGWAGGNAKS KSLFKNCDLM WKQSIWTSTI SSHLATKHLK EGGLLTLAGA KAALDGTPGM IGYGMAKGAV HQLCQSLAGK NSGMPPGAAA IAVLPVTLDT PMNRKSMPEA DFSSWTPLEF LVETFHDWIT GKNRPSSGSL IQVVTTEGRT ELTPAYF
 OMIM    261630
 3D Structure    ModBase 3D Structure for P09417
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 Host    E Coli
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 Purity/Purification    >=90%
 Form/Format    1 mg/ml solution in 20 mM Tris-HCl buffer (pH 8.0) containing 10% glycerol and 2 mM DTT
Appearance: Liquid
 Concentration    1 mg/ml (lot specific)
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 Biological Activity    Specific activity: > 27 units/ml One unit will oxidize 1.0 umole of b-NADH to b-NAD with 6,7-Dimethyldihydropterine (quinonoid isomer) as the non-nucleotide substrate per minute at pH 7.2 at 25 degree C
 Results    Specific activity: > 27 units/ml
 Unit Definition    One unit will oxidize 1.0 umole of b-NADH to b-NAD with 6,7-Dimethyldihydropterine (quinonoid isomer) as the non-nucleotide substrate per minute at pH 7.2 at 25 degree C
 Handling    Centrifuge the vial prior to opening.
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 Preparation and Storage    At -20 degree C
Shelf Life: 12 months
 Other Notes    Small volumes of QDPR recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for QDPR recombinant protein

   Background: QDPR is a member of the short-chain dehydrogenases/reductase (SDR) family of enzymes. Functioning as a homodimer, QDPR plays an important role in the recycling of tetrahydrobiopterin (BH4), an essential cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine). More specifically, QDPR catalyzes the regeneration of BH4 from quinonoid dihydrobiopterin (qBH2), the product generated from the hydroxylation reactions. Mutations in the gene encoding QDPR can lead to phenylketonuria II. Recombinant human QDPR protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.
 Product Categories/Family for QDPR recombinant protein    Proteins and Enzymes; Proteins and Enzymes (A-Z)
 Applications Tested/Suitable for QDPR recombinant protein   

SDS-PAGE

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 Testing Data of QDPR recombinant protein    QDPR recombinant protein Testing Data image
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NCBI/Uniprot data below describe general gene information for QDPR. It may not necessarily be applicable to this product.
 NCBI GI #    208973246
 NCBI GeneID    5860
 NCBI Accession #    NP_000311.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000320.2 [Other Products]
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 UniProt Primary Accession #    P09417 [Other Products]
 UniProt Secondary Accession #    Q53F52; Q9H3M5; A8K158; B3KW71 [Other Products]
 UniProt Related Accession #    P09417 [Other Products]
 Molecular Weight    28.2 kDa (267 aa, 1-244 aa + His Tag)
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 NCBI Official Full Name    dihydropteridine reductase isoform 1
 NCBI Official Synonym Full Names    quinoid dihydropteridine reductase
 NCBI Official Symbol    QDPR [Similar Products]
 NCBI Official Synonym Symbols   
DHPR; PKU2; SDR33C1
[Similar Products]
 NCBI Protein Information    dihydropteridine reductase
 UniProt Protein Name    Dihydropteridine reductase
 UniProt Synonym Protein Names   
HDHPR; Quinoid dihydropteridine reductase; Short chain dehydrogenase/reductase family 33C member 1
 Protein Family    Dihydropteridine reductase
 UniProt Gene Name    QDPR [Similar Products]
 UniProt Synonym Gene Names    DHPR; SDR33C1 [Similar Products]
 UniProt Entry Name    DHPR_HUMAN
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 NCBI Summary for QDPR    This gene encodes the enzyme dihydropteridine reductase, which catalyzes the NADH-mediated reduction of quinonoid dihydrobiopterin. This enzyme is an essential component of the pterin-dependent aromatic amino acid hydroxylating systems. Mutations in this gene resulting in QDPR deficiency include aberrant splicing, amino acid substitutions, insertions, or premature terminations. Dihydropteridine reductase deficiency presents as atypical phenylketonuria due to insufficient production of biopterin, a cofactor for phenylalanine hydroxylase. [provided by RefSeq, Jul 2008]
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 UniProt Comments for QDPR    QDPR: The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases. Defects in QDPR are the cause of BH4-deficient hyperphenylalaninemia type C (HPABH4C); 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. Belongs to the short-chain dehydrogenases/reductases (SDR) family.

Protein type: Oxidoreductase; EC 1.5.1.34; Cofactor and Vitamin Metabolism - folate biosynthesis

Chromosomal Location of Human Ortholog: 4p15.31

Cellular Component: neuron projection; mitochondrion; cytoplasm; cytosol

Molecular Function: protein homodimerization activity; electron carrier activity; 6,7-dihydropteridine reductase activity

Biological Process: amino acid metabolic process; tetrahydrobiopterin biosynthetic process; L-phenylalanine catabolic process; response to glucagon stimulus; dihydrobiopterin metabolic process; response to lead ion; liver development; response to aluminum ion

Disease: Hyperphenylalaninemia, Bh4-deficient, C
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 Research Articles on QDPR    1. The mutation spectrum of the QDPR gene is different in the Chinese population. Most mutations are related to severe phenotype.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with QDPR recombinant proteinPathways associated with QDPR recombinant protein
 Reference Product  PubMed Publications
 PAH recombinant protein  >6 publications with QDPR and PAH
 PCBD1 recombinant protein  >4 publications with QDPR and PCBD1
 SPR recombinant protein  >3 publications with QDPR and SPR
 Products by Pathway  Pathway Diagram
 Folate Biosynthesis Pathway antibodies  Folate Biosynthesis Pathway Diagram
 Folate Biosynthesis Pathway antibodies  Folate Biosynthesis Pathway Diagram
 L-phenylalanine Degradation I (aerobic) Pathway antibodies  L-phenylalanine Degradation I (aerobic) Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
 Phenylalanine And Tyrosine Catabolism Pathway antibodies  Phenylalanine And Tyrosine Catabolism Pathway Diagram
 Phenylalanine Degradation/tyrosine Biosynthesis Pathway antibodies  Phenylalanine Degradation/tyrosine Biosynthesis Pathway Diagram
Diseases associated with QDPR recombinant proteinOrgans/Tissues associated with QDPR recombinant protein
 Disease Name  Pubmed Publications
 Phenylketonurias Antibodies  >11 publications with QDPR and Phenylketonurias
 HYPERPHENYLALANINEMIA, BH4-DEFICIENT, C Antibodies  >11 publications with QDPR and HYPERPHENYLALANINEMIA, BH4-DEFICIENT, C
 Cognition Disorders Antibodies  >4 publications with QDPR and Cognition Disorders
 Atrophy Antibodies  >1 publications with QDPR and Atrophy
 Fibrosis Antibodies  >1 publications with QDPR and Fibrosis
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >8 publications with QDPR and Blood
 Brain Antibodies  >6 publications with QDPR and Brain
 Kidney Antibodies  >2 publications with QDPR and Kidney
 Nerve Antibodies  >1 publications with QDPR and Nerve
 Lung Antibodies  >1 publications with QDPR and Lung
 Liver Antibodies  >1 publications with QDPR and Liver
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