Quantity size:
0.2ml
Concentration:
1mg/ml 半乳糖脑苷脂酶抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.半乳糖脑苷脂酶抗体 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. 半乳糖脑苷脂酶抗体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.
半乳糖脑苷脂酶抗体Also known as:
Gacy; Galc; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; Galc; GALCERase; Twitcher.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, .
●
Immunogen: KLH conjugated synthetic peptide derived from human Galc.
●
Predicted Molecular Weight: 71kDa.
半乳糖脑苷脂酶抗体Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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企业名称
上海康朗生物科技有限公司
企业信息已认证
企业类型
信用代码
310112001510759
成立日期
2015-05-29
注册资本
100
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从事生物科技技术领域内技术开发、技术咨询、技术服务、技术转让,实验分析仪器、化工产品及原料(除危险化学品、监控化学品、烟花爆竹、民用爆竹物品、易制毒化学品)、仪器仪表、环保设备、酒店用品、皮革制品、床上用品、电子产品的销售。【依法须经批准的项目,经相关部门批准后方可开展经营活动】
上海康朗生物科技有限公司
公司地址
上海市闵行区闵北路88弄1-30号第22栋BV145室
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