FITC标记的次黄嘌呤磷酸核糖基转移酶1抗体-抗体-抗体-生物在线
FITC标记的次黄嘌呤磷酸核糖基转移酶1抗体

FITC标记的次黄嘌呤磷酸核糖基转移酶1抗体

商家询价

产品名称: FITC标记的次黄嘌呤磷酸核糖基转移酶1抗体

英文名称: Anti-HPRT/FITC

产品编号: HZ-9026R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: IF=1:50-200

上海沪震实业有限公司
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 Rabbit Anti-HPRT/FITC Conjugated antibody 

FITC标记的次黄嘌呤磷酸核糖基转移酶1抗体

 

产品编号 bs-9026R-FITC
英文名称 Anti-HPRT/FITC
中文名称 FITC标记的次黄嘌呤磷酸核糖基转移酶1抗体
别    名 HGPRT; HGPRTase; HPRT 1; HPRT_HUMAN; HPRT1; Hypoxanthine guanine phosphoribosyltransferase; Hypoxanthine phosphoribosyltransferase 1 (Lesch Nyhan syndrome); Hypoxanthine phosphoribosyltransferase 1; Hypoxanthine-guanine phosphoribosyltransferase; HPRT_HUMAN.  
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  免疫学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 24kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HPRT
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:
The protein encoded by this gene is a transferase, which catalyzes conversion of hypoxanthine to inosine monophosphate and guanine to guanosine monophosphate via transfer of the 5-phosphoribosyl group from 5-phosphoribosyl 1-pyrophosphate. This enzyme plays a central role in the generation of purine nucleotides through the purine salvage pathway. Mutations in this gene result in Lesch-Nyhan syndrome or gout.[provided by RefSeq, Jun 2009].

Function:
Converts guanine to guanosine monophosphate, and hypoxanthine to inosine monophosphate. Transfers the 5-phosphoribosyl group from 5-phosphoribosylpyrophosphate onto the purine. Plays a central role in the generation of purine nucleotides through the purine salvage pathway.

Subunit:
Homotetramer.

Subcellular Location:
Cytoplasm.

DISEASE:
Defects in HPRT1 are the cause of Lesch-Nyhan syndrome (LNS) [MIM:300322]. LNS is characterized by complete lack of enzymatic activity that results in hyperuricemia, choreoathetosis, mental retardation, and compulsive self-mutilation. 
Defects in HPRT1 are the cause of gout HPRT-related (GOUT-HPRT) [MIM:300323]; also known as HPRT-related gout or Kelley-Seegmiller syndrome. Gout is characterized by partial enzyme activity and hyperuricemia.

Similarity:
Belongs to the purine/pyrimidine phosphoribosyltransferase family.

Database links:

Entrez Gene: 395653 Chicken

Entrez Gene: 3251 Human

Entrez Gene: 15452 Mouse

Entrez Gene: 24465 Rat

Omim: 308000 Human

SwissProt: Q9W719 Chicken

SwissProt: P00494 Chinese Hamster

SwissProt: P00492 Human



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

由该基因编码的蛋白质是转移酶,通过5-磷酸核糖基焦磷酸转移5-磷酸核糖基,催化次黄嘌呤转化为肌苷酸和鸟嘌呤到鸟苷酸一磷酸。这种酶在嘌呤核苷酸产生的嘌呤补救途径中发挥核心作用。该基因的突变导致LaskyNhann综合征或痛风。[由RefSeq,Jun 2009提供。