|Application ||WB, E|
|Clone Names||9G7F12; 7G6D7|
|Calculated MW||45419 Da|
|Description||Lysosomal acid lipase (LAL), with 378-amino acid protein( 43-54 kDa), functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides which are taken up by receptor-mediated endocytosis. An inherited deficiency or low activity of human lysosomal acid lipase results in the intralysosomal storage of the respective lipid substrates. So it is also responsible for the rare conditions of Wolman disease and cholesteryl ester storage disease (CESD). As the enzyme is synthesized by all nucleated cells, lipid-laden cells are found in all organs, particularly in liver, spleen, the adrenal and the hemopoietic system, and in the intestine as well as in the lymph nodes, lungs, testes, and ovaries.|
|Immunogen||Purified recombinant fragment of LAL expressed in E. Coli.|
|Formulation||Purified antibody in PBS containing 0.03% sodium azide.|
|Other Names||Lysosomal acid lipase/cholesteryl ester hydrolase, Acid cholesteryl ester hydrolase, LAL, 22.214.171.124, Cholesteryl esterase, Lipase A, Sterol esterase, LIPA|
|Dilution||WB~~1/500 - 1/2000|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||LAL Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|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.|
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1. Uta Drebber, Matthias Andersen, Hans U Kasper, et al, World J Gastroenterol. 2005 Apr 21;11(15):2364-6. 2. Renata Boldrini, Rita Devito, R.Biselli,et al, Pathol Res Pract. 2004;200(3):231-40.
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