| 克隆類型 ： |
Rabbit Polyclonal IgG |
|
免 疫 原 ： |
Peptide derived from Hu Galc. |
|
純化方式 ： |
Immunogen affinity purified |
|
緩 沖 液 ： |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
產(chǎn)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. |
| 適用物種 ： |
Hu, Mo, Rat, Dog, Pig, Cow, Hor, Guinea Pig, |
| 應(yīng)用說明 ： |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 別 名 ： |
Gacy; Galc; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; Galc; GALCERase; Twitcher. |
| 保存條件 ： |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 標(biāo) 記 物 ： |
PLLABS公司可供應(yīng)生物素、辣根過氧化物酶、熒光素標(biāo)記抗體 |
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產(chǎn)品說明 ： |
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