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LAL

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Mouse Antibody to LAL
Catalog#   :  20061
Size/Concentration: 50ul100μl
Price: 12802180
Instruction:
Specification
Entrez GeneID3988
AliasesLAL; CESD; LIPA
Clone#9G7F12
Host/IsotypeMouse IgG2a
Storage4℃; -20℃ for long term storage. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenPurified recombinant fragment of LAL expressed in E. Coli.
FormulationAscitic fluid containing 0.03% sodium azide.
Application
ELISA1/10000
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.
References
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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