产品名称： Anti-Galactocerebrosidase/FITC Conjugated抗体
级 别： , 分析纯, 见说明书
价 格： 2980元
抗原： KLH conjugated synthetic peptide derived from human Galactocerebroside
适用物种： Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig,
更新时间： 2019/4/16 10:20:00
|别 名||Gacy; Galc; Galactocerebroside beta galactosidase; Galactosylceramide beta-galactosidase; Galc; GALCERase; Twitcher; GALC_HUMAN; Galactocerebroside.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
|研究领域||肿瘤 心血管 细胞生物 神经生物学|
|交叉反应||Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig,|
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
|分 子 量||71kDa|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human Galactocerebroside|
|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.|
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.
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.
Highest level of activity in testes compared to brain, kidney, placenta and liver. Can also be found in urine.
Belongs to the glycosyl hydrolase 59 family.
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.