LIPA
Reactivité: Humain, Rat, Cheval, Porc, Boeuf (Vache), Lapin, Mouton
WB
Hôte: Lapin
Polyclonal
unconjugated
Indications d'application
Western Blot: Dilution 1: 200- 1: 1,000 ELISA: Propose dilution 1: 10,000. Determining optimal working dilutions by titration test.
Restrictions
For Research Use only
Stock
-20 °C
Drebber, Andersen, Kasper, Lohse, Stolte, Dienes: "Severe chronic diarrhea and weight loss in cholesteryl ester storage disease: a case report." dans: World journal of gastroenterology : WJG, Vol. 11, Issue 15, pp. 2364-6, (2005) (PubMed).
Boldrini, Devito, Biselli, Filocamo, Bosman: "Wolman disease and cholesteryl ester storage disease diagnosed by histological and ultrastructural examination of intestinal and liver biopsy." dans: Pathology, research and practice, Vol. 200, Issue 3, pp. 231-40, (2004) (PubMed).
Antigène
Lipase A (LIPA)
(Lipase A, Lysosomal Acid, Cholesterol Esterase (LIPA))
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.