产品简要
公司名称 :
MyBioSource
产品类型 :
蛋白
产品名称 :
Recombinant Human Quinoid Dihydropteridine Reductase
目录 :
MBS144753
规格 :
0.005毫克
价格 :
140美元
更多信息或购买 :
产品信息
目录号 :
MBS144753
产品类型 :
重组蛋白
产品全称 :
Recombinant Human Quinoid Dihydropteridine Reductase
产品简称 :
Quinoid Dihydropteridine Reductase
产品名称同义词 :
QDPR Human; Quinoid Dihydropteridine Reductase Human Recombinant; Dihydropteridine reductase; HDHPR; Quinoid dihydropteridine reductase; QDPR; DHPR; PKU2; SDR33C1
其他名称 :
dihydropteridine reductase; Dihydropteridine reductase; dihydropteridine reductase; 6,7-dihydropteridine reductase; HDHPR; short chain dehydrogenase/reductase family 33C, member 1; quinoid dihydropteridine reductase; HDHPR; Quinoid dihydropteridine reductase
产品基因名称 :
QDPR
其他基因名称 :
QDPR;QDPR;DHPR;PKU2;SDR33C1;DHPR
UniProt数据库进入名 :
DHPR_HUMAN
宿主 :
大肠杆菌
序列长度 :
244
纯度 :
Greater than 90.0% as determined by SDS-PAGE.
形式 :
QDPR protein solution (1mg/ml) containing 20mM Tris-HCl buffer (pH 8.0), 10% glycerol and 2mM DTT. Sterile Filtered colorless solution.
产品种类 :
ENZYMES;酶;还原酶
产品描述 :
Description: QDPR Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 267 amino acids (1-244 a.a.) and having a molecular mass of 28.2kDa.QDPR is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. Introduction: QDPR belongs to the short-chain dehydrogenases/reductase (SDR) family of enzymes. Operating as a homodimer, QDPR has an imperative role in the recycling of tetrahydrobiopterin (BH4), a vital cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine). More precisely, QDPR catalyzes the regeneration of BH4 from quinonoid dihydrobiopterin (qBH2), the product generated from the hydroxylation reactions. Mutations in the QDPR gene may lead to phenylketonuria II.
NCBI GI登录号 :
208973246
NCBI登录号 :
NP_000311.2
NCBI基因登录号 :
NM_000320.2
UniProt数据库登录号 :
P09417
NCBI分子量 :
22,408 Da
NCBI信号通路 :
Folate Biosynthesis Pathway (83018); Folate Biosynthesis Pathway (404); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Phenylalanine And Tyrosine Catabolism Pathway (106189); Phenylalanine Degradation I (aerobic) Pathway (139195); Phenylalanine Degradation/tyrosine Biosynthesis Pathway (142375)
NCBI总结 :
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]
UniProt数据库总结 :
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: EC 1.5.1.34; Oxidoreductase; 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
尺寸1 :
0.005毫克
价格1 :
140美元
尺寸2 :
0.02毫克
价格2 :
205
size3 :
1毫克
价格3 :
3490
更多信息或购买 :
公司信息
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MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com1-858-633-0165
公司总部: 美国
MyBioSource,LLC最初由三名热情澎湃的提供高品质试剂的科学家联合创立于温哥华,公司愿景是“生物研究试剂的源头”,现在位于圣地亚哥市。