蚕顿笔搁蛋白抗体
规格：1尘驳/1尘濒
英文名： QDPR
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, membe
分子量： 26kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human QDPR
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep,
细胞定位：
蚕顿笔搁蛋白抗体产物介绍：background: 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,蚕顿笔搁蛋白抗体 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蚕顿笔搁蛋白抗体. HPABH4C is lethal if untreated. Similarity: Belongs to the short-chain dehydrogenases/reductases (SDR) family. 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.
蚕顿笔搁蛋白抗体产物应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：细胞生物 神经生物学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid