Lipase A, Lysosomal Acid, Cholesterol Esterase (LIPA) antibody

Details for Product No. ABIN966473
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Antigen
Synonyms cesd, lal, LIPA, CESD, LAL, Chole, Chole2, Lip1, AA960673, Lal, Lip-1
Reactivity
Human
(35), (4), (2)
Host
Please inquire
(21), (15)
Clonality
Monoclonal
Conjugate
Un-conjugated
(2), (2), (2)
Application
Western Blotting (WB), ELISA
(30), (19), (3), (3), (2), (2), (2), (1), (1)
Pubmed 2 references available
Quantity 0.1 mg
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Catalog No. ABIN966473
371.25 $
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Isotype IgG1
Specificity Ni-NTA purified truncated recombinant LAL expressed in E. Coli strain BL21 (DE3)
Purification Antibodies are purified by protein A affinity chromatography
Alternative Name LAL
Background 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.
Application Notes 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
Storage -20 °C
Product cited in: Boldrini, Devito, Biselli et al.: "Wolman disease and cholesteryl ester storage disease diagnosed by histological and ultrastructural examination of intestinal and liver biopsy." in: Pathology, research and practice, Vol. 200, Issue 3, pp. 231-40, 2004 (PubMed).

Drebber, Andersen, Kasper et al.: "Severe chronic diarrhea and weight loss in cholesteryl ester storage disease: a case report." in: World journal of gastroenterology : WJG, Vol. 11, Issue 15, pp. 2364-6, 2005 (PubMed).

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