Rabbit Anti-Galactocerebrosidase antibody |
反应物种(预测) |
Human,Mouse,Dog,Pig,Cow,Horse,Rabbit,GuineaPig |
产品应用(已验证) |
WB |
产品应用(可尝试) |
ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000, |
研究领域 |
肿瘤,心血管,细胞生物,神经生物学 |
标签 |
Array |
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Sample:
Brain (Rat) Lysate at 40 ug
Liver (Rat) Lysate at 40 ug
Primary: Anti-Galactocerebroside (bs-4691R) at 1/300 dilution
Secondary: HRP conjugated Goat-Anti-rabbit IgG (bs-0295G-HRP) at 1/5000 dilution
Predicted band size: 71 kD
Observed band size: 71 kD
RRID:AB_11104598
产品名称:Rabbit Anti-Galactocerebrosidase antibody
别名: Gacy; Galc; Galactocerebroside beta galactosidase; Galactosylceramide beta-galactosidase; Galc; GALCERase; Twitcher; GALC_HUMAN; Galactocerebroside.
中文名称:半乳糖脑苷脂酶抗体
英文名称:Rabbit Anti-Galactocerebrosidase antibody
抗体来源: Rabbit
克隆类型:多克隆
细胞定位:细胞浆
性 状:Liquid
亚 型:IgG
纯化方法:affinity purified by Protein A
保存条件:Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
免 疫 原:KLH conjugated synthetic peptide derived from human Galactocerebroside
抗原表位:301-400/685
SWISS:P54803
Gene ID :2581
Human Gene ID:2581
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, kidn
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.