LAL Monoclonal Antibody

    • 货号:YM0410
    • 应用:WB;ELISA
    • 种属:Human
      • 靶点:
      • LAL
      • 简介:
      • >>Steroid biosynthesis;>>Lysosome;>>Cholesterol metabolism
      • 基因名称:
      • LIPA
      • 蛋白名称:
      • Lysosomal acid lipase/cholesteryl ester hydrolase
      • Human Gene Id:
      • 3988
      • Human Swiss Prot No:
      • P38571
      • Mouse Swiss Prot No:
      • Q9Z0M5
      • 免疫原:
      • Purified recombinant fragment of LAL expressed in E. Coli.
      • 特异性:
      • LAL Monoclonal Antibody detects endogenous levels of LAL protein.
      • 组成:
      • Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
      • 来源:
      • Monoclonal, Mouse
      • 稀释:
      • WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.
      • 纯化工艺:
      • Affinity purification
      • 浓度:
      • 1 mg/ml
      • 储存:
      • -15°C to -25°C/1 year(Do not lower than -25°C)
      • 其他名称:
      • LIPA;Lysosomal acid lipase/cholesteryl ester hydrolase;Acid cholesteryl ester hydrolase;LAL;Cholesteryl esterase;Lipase A;Sterol esterase
      • 分子量:
      • 45kD
      • 背景:
      • 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],
      • 功能:
      • catalytic activity:A steryl ester + H(2)O = a sterol + a fatty acid.,disease: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.,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 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.,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 s
      • 细胞定位:
      • Lysosome .
      • 组织表达:
      • Most abundantly expressed in brain, lung, kidney and mammary gland, a moderate expression seen in placenta and expressed at low levels in the liver and heart.
      • 产品图片
      • Western Blot analysis using LAL Monoclonal Antibody against LAL recombinant protein.