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Rabbit Anti-QDPR antibody
Rabbit Anti-QDPR antibody
6,7 dihydropteridine reductase; DHPR; DHPR_HUMAN; Dihydropteridine reductase; HDHPR; HPR; PKU2; Qdpr; Quinoid dihydropteridine reductase; SDR33C1; Short chain dehydrogenase/reductase family 33C, member 1.
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  • NO.:SL7489R
    Clonality:Polyclonal
    Immunogen Species:Rabbit
    React Species:Rat,(predicted: Human,Mouse,Dog,)
    Applications:ELISA IHC-P IHC-F ICC IF
    concentration:1mg/ml
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Details

Product Name QDPR
Chinese Name QDPR蛋白抗体
Alias 6,7 dihydropteridine reductase; DHPR; DHPR_HUMAN; Dihydropteridine reductase; HDHPR; HPR; PKU2; Qdpr; Quinoid dihydropteridine reductase; SDR33C1; Short chain dehydrogenase/reductase family 33C, member 1.  
Research Area Cell biology  Neurobiology  Signal transduction  
Immunogen Species Rabbit
Clonality Polyclonal
React Species Rat,  (predicted: Human, Mouse, Dog, )
Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight 26kDa
Cellular localization cytoplasmic Extracellular matrix 
Form Liquid
Concentration 1mg/ml
immunogen KLH conjugated synthetic peptide derived from human QDPR: 141-244/244 
Lsotype IgG
Purification affinity purified by Protein A
Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
Product Detail 6,7 dihydropteridine reductase; DHPR; DHPR_HUMAN; Dihydropteridine reductase; HDHPR; HPR; PKU2; Qdpr; Quinoid dihydropteridine reductase; SDR33C1; Short chain dehydrogenase/reductase family 33C, member 1.

Function:
The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases.

Subunit:
Homodimer.

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.

Similarity:
Belongs to the short-chain dehydrogenases/reductases (SDR) family.

SWISS:
P09417

Gene ID:
5860

Database links:

Entrez Gene: 5860 Human

Entrez Gene: 110391 Mouse

Entrez Gene: 64192 Rat

Omim: 612676 Human

SwissProt: P09417 Human

SwissProt: Q8BVI4 Mouse

SwissProt: P11348 Rat

Unigene: 75438 Human

Unigene: 30204 Mouse

Unigene: 241 Rat



Product Picture
Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (QDPR) Polyclonal Antibody, Unconjugated (SL7489R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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