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（Formulation）
Purified antibody in PBS containing 0.03% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
纯化工艺（Immunogen）
Affinity purification
背景（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],
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
蛋白名称
Lysosomal acid lipase/cholesteryl ester hydrolase
其他名称
LIPA; Lysosomal acid lipase/cholesteryl ester hydrolase; Acid cholesteryl ester hydrolase; LAL; Cholesteryl esterase; Lipase A; Sterol esterase
Fields
>>Steroid biosynthesis;>>Lysosome;>>Cholesterol metabolism
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.
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