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產(chǎn)品中心-北京博奧森生物技術(shù)有限公司
Rabbit Anti-Galactocerebrosidase/AP Conjugated antibody (bs-4691R-AP)
~~~促銷,代碼KT202502A~~~
~~~促銷,代碼KT202502B~~~
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-4691R-AP
英文名稱 Rabbit Anti-Galactocerebrosidase/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標(biāo)記的半乳糖腦苷脂酶抗體
別    名 Gacy; Galc; Galactocerebroside beta galactosidase; Galactosylceramide beta-galactosidase; Galc; GALCERase; Twitcher; GALC_HUMAN; Galactocerebroside.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  心血管  細(xì)胞生物  神經(jīng)生物學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Rat,  (predicted: Human, Mouse, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, )
產(chǎn)品應(yīng)用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 71kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Galactocerebroside
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
Galactosylceramidase hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is an enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. It shows highest level of activity in testes compared to brain, kidney, placenta and liver. It can also be found in urine. Defects in Galactosylceramidase are the cause of globoid cell leukodystrophy (GLD); also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified.

Function:
Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.

Subcellular Location:
Lysosomal.

Tissue Specificity:
Highest level of activity in testes compared to brain, kidney, placenta and liver. Can also be found in urine.

Similarity:
Belongs to the glycosyl hydrolase 59 family.

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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