胆固醇酯水解酶抗体 Lysosomal acid lipase

胆固醇酯水解酶抗体

规格：1mg/1ml

英文名： Lysosomal acid lipase

别名： Acid Cholesteryl Ester Hydrolase; CESD; cholesterol ester hydrolase; cholesterol ester storage disease; Cholesteryl Esterase; Hydrolase deficiency; LAL; LAL deficiency cholesterol ester; LICH_HUMAN; L

分子量： 43kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human Lysosoma

交叉反应：Human, Mouse, Rat, Cow, Horse, Sheep,

细胞定位：

胆固醇酯水解酶抗体产品介绍：background: This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jan 2014] Function: Crucial for the intracellular hydrolysis of cholesteryl esters and triglycerides that have been internalized via receptor-mediated endocytosis of lipoprotein particles. Important in mediating the effect of LDL (low density lipoprotein) uptake on suppression of hydroxymethylglutaryl-CoA reductase and activation of endogenous cellular cholesteryl ester formation. Subcellular Location: Lysosome. DISEASE: Defects in LIPA are the cause of Wolman disease (WOD) [MIM:278000]. WOD is a severe manifestation of LIPA deficiency, leading to the accumulation of 胆固醇酯水解酶抗体cholesteryl esters and triglycerides in most tissues of the body. WOD occurs in infancy and is nearly always fatal before the age of 1 year. Defects in LIPA are the cause of cholesteryl ester storage disease (CESD) [MIM:278000]. CESD is a mild manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. It is characterized by late-onset. Similarity: Belongs to the胆固醇酯水解酶抗体 AB hydrolase superfamily. Lipase family. Gene ID: 3988 Database links: Entrez Gene: 3988 Human Omim: 278000 Human SwissProt: P38571 Human Unigene: 643030 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产胆固醇酯水解酶抗体品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究领域：肿瘤  细胞生物  信号转导  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid