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

Details for Product No. ABIN969484
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Antigen
Synonyms cesd, lal, LIPA, CESD, LAL, Chole, Chole2, Lip1, AA960673, Lal, Lip-1
Reactivity
Human
(35), (4), (2)
Host
Mouse
(21), (14)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(2), (2), (2)
Application
ELISA, Western Blotting (WB)
(30), (19), (3), (3), (2), (2), (2), (1), (1)
Pubmed 2 references available
Quantity 100 μL
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Catalog No. ABIN969484
319.00 $
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Immunogen Purified recombinant fragment of LAL expressed in E. coli.
Clone 9G7F12
Isotype IgG2a
Purification Purified
Alternative Name LAL
Background Synonyms:
LAL, CESD, LIPA
Lysosomal acid lipase (LAL), with 378 AA 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.
Gene ID 3988
Application Notes Recommended Dilution:
ELISA: 1/10000, WB: 1/500 - 1/2000
Not yet tested in other applications.
Determining optimal working dilutions by titration test.
Restrictions For Research Use only
Format Liquid
Buffer PBS containing 0.03 % sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Store at 4 °C or at -20 °C for long term.
Supplier Images
anti-Lipase A, Lysosomal Acid, Cholesterol Esterase (LIPA) antibody Figure 1: Western blot analysis using LAL mouse mAb against LAL recombinant protein.
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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