产品编号 | bs-7489R-PE-Cy7 |
英文名称 | Anti-QDPR/PE-Cy7 |
中文名称 | PE-Cy7标记的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. |
规格价格 | 100ul/2980元 购买 大包装/询价 |
说 明 书 | 100ul |
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 | (predicted: Human, Mouse, Rat, Dog, ) |
产品应用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 26kDa |
性 状 | Lyophilized or Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human QDPR |
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 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
产品介绍 |
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, 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. Database links: Entrez Gene: 5860 Human Entrez Gene: 110391 Mouse Omim: 612676 Human SwissProt: P09417 Human SwissProt: Q8BVI4 Mouse 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. |
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |