| 克隆类型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from Hu Galc. |
|
纯化方式 ： |
Immunogen affinity purified |
|
缓 冲 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
产品描述 ： |
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. |
| 适用物种 ： |
Hu, Mo, Rat, Dog, Pig, Cow, Hor, Guinea Pig, |
| 应用说明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 别 名 ： |
Gacy; Galc; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; Galc; GALCERase; Twitcher. |
| 保存条件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 标 记 物 ： |
PLLABS公司可供应生物素、辣根过氧化物酶、荧光素标记抗体 |
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产品说明 ： |
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