货号 |
bs-4691R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-Galactocerebrosidase antibody |
中文名称 |
Rabbit Anti-Galactocerebrosidase antibody |
研究领域 |
肿瘤,心血管,细胞生物,神经生物学 |
英文别名 |
Gacy; Galc; Galactocerebroside beta galactosidase; Galactosylceramide beta-galactosidase; Galc; GALCERase; Twitcher; GALC_HUMAN; Galactocerebroside. |
反应物种(已验证) |
Rat |
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(可推荐) |
ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
71 |
细胞定位 |
细胞浆 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human Galactocerebroside |
抗原表位 |
301-400/685 |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Lysosomal. |
Tissue |
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. |
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 |
SWISS |
P54803 |
Gene ID |
2581 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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. |