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墨蝶蛉还原酶抗体
  • 产品货号:
    BN40329R
  • 中文名称:
    墨蝶蛉还原酶抗体
  • 英文名称:
    Rabbit anti-SPR Polyclonal antibody
  • 货号

    产品规格

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  • BN40329R-100ul

    100ul

    ¥2360.00

    交叉反应:Human,Mouse,Rat(predicted:Pig,Horse,Rabbit) 推荐应用:WB,IHC-P,IHC-F,ICC,IF,ELISA

  • BN40329R-200ul

    200ul

    ¥3490.00

    交叉反应:Human,Mouse,Rat(predicted:Pig,Horse,Rabbit) 推荐应用:WB,IHC-P,IHC-F,ICC,IF,ELISA

英文名称SPR
中文名称墨蝶蛉还原酶抗体
别    名SDR38C1; Sepiapterin reductase (7,8 dihydrobiopterin:NADP+ oxidoreductase); Sepiapterin reductase; Short chain dehydrogenase/reductase family 38C, member 1; SPR; SPRE_HUMAN.  
研究领域肿瘤  细胞生物  神经生物学  信号转导  新陈代谢  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat,  (predicted: Pig, Horse, Rabbit, )
产品应用WB=1:500-2000 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28kDa
细胞定位细胞浆 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Sepiapterin reductase:101-200/261 
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.
PubMedPubMed
产品介绍This gene encodes an aldo-keto reductase that catalyzes the NADPH-dependent reduction of pteridine derivatives and is important in the biosynthesis of tetrahydrobiopterin (BH4). Mutations in this gene result in DOPA-responsive dystonia due to sepiaterin reductase deficiency. A pseudogene has been identified on chromosome 1. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.

Subunit:
Homodimer.

Subcellular Location:
Cytoplasm.

Post-translational modifications:
In vitro phosphorylation of Ser-213 by CaMK2 does not change kinetic parameters.

DISEASE:
Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD) [MIM:612716]. In the majority of cases, patients manifest progressive psychomotor retardation, dystonia and spasticity. Cognitive anomalies are also often present. The disease is due to severe dopamine and serotonin deficiencies in the central nervous system caused by a defect in BH4 synthesis. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures.

Similarity:
Belongs to the sepiapterin reductase family.

SWISS:
P35270

Gene ID:
6697

Database links:

Entrez Gene: 6697 Human

Omim: 182125 Human

SwissProt: P35270 Human

Unigene: 301540 Human



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


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