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Rabbit Anti-APRT/FITC Conjugated antibody (bs-12506R-FITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-12506R-FITC
英文名稱 Rabbit Anti-APRT/FITC Conjugated antibody
中文名稱 FITC標(biāo)記的腺嘌呤磷酸核糖轉(zhuǎn)移酶抗體
別    名 Adenine phosphoribosyltransferase; AMP; AMP diphosphorylase; AMP pyrophosphorylase; APRT; APT_HUMAN; DKFZp686D13177; MGC125856; MGC125857; MGC129961; Transphosphoribosidase.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Sheep, )
產(chǎn)品應(yīng)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 19kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human APRT
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
APRT is a 180 amino acid protein that localizes to the cytoplasm and belongs to the purine/pyrimidine phosphoribosyltransferase family. Existing as a homodimer, APRT functions to catalyze the formation of inorganic pyrophosphate and AMP from adenine and 5-phosphoribosyl-1-pyrophosphate (PRPP), a reaction that is essential for both purine metabolism and AMP biosynthesis. Defects in the gene encoding APRT are the cause of APRT deficiency, also known as 2,8-dihydroxyadenine urolithiasis, which is an autosomal recessive disease that results in renal failure. The gene encoding APRT maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome. The GAN gene is located on chromosome 16 and, with mutation, may lead to giant axonal neuropathy, a nervous system disorder characterized by increasing malfunction with growth. The rare disorder Rubinstein-Taybi syndrome is also associated with chromosome 16, as is Crohn's disease, which is a gastrointestinal inflammatory condition.

Function:
Catalyzes a salvage reaction resulting in the formation of AMP, that is energically less costly than de novo synthesis.

Subunit:
Homodimer.

Subcellular Location:
Cytoplasm.

DISEASE:
Defects in APRT are the cause of adenine phosphoribosyltransferase deficiency (APRTD) [MIM:102600]; also known as 2,8-dihydroxyadenine urolithiasis. An enzymatic deficiency that can lead to urolithiasis and renal failure. Patients have 2,8-dihydroxyadenine (DHA) urinary stones.

Similarity:
Belongs to the purine/pyrimidine phosphoribosyltransferase family.

Database links:

Entrez Gene: 353 Human

Entrez Gene: 292072 Rat

Omim: 102600 Human

SwissProt: P07741 Human

SwissProt: P36972 Rat

Unigene: 28914 Human

Unigene: 2498 Rat



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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