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Rabbit Anti-QDPR antibody (bs-7489R)
~~~促销,代码KT001~~~
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说明书: 100ul  200ul
100ul/1680.00元
200ul/2480.00元
大包装/询价

产品编号 bs-7489R
英文名称 QDPR
中文名称 QDPR蛋白抗体
别    名 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.  
研究领域 细胞生物  神经生物学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,  (predicted: Human, Mouse, Dog, )
产品应用 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 26kDa
细胞定位 细胞浆 细胞外基质 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human QDPR:141-244/244 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed PubMed
产品介绍 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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片
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 (bs-7489R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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