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

Antigen: Lipase A, Lysosomal Acid, Cholesterol Esterase (LIPA)

Synonyms: LAL, CESD, Lip1, Chole, Chole2, LIPA, lal, cesd, MGC97855, MGC130898, MGC159953

Clonality: Monoclonal

Host: Please enquire

Reactivity: Human

Application: Western Blotting (WB), ELISA

Catalog no.: ABIN966473

Additional Information

Alternative name: LAL

Isotype: IgG1

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.

Specificity: Ni-NTA purified truncated recombinant LAL expressed in E. Coli strain BL21 (DE3)

Application Details

Application Notes: Western Blot: Dilution 1: 200- 1: 1,000
ELISA: Propose dilution 1: 10,000.
Determining optimal working dilutions by titration test.

Purification: Antibodies are purified by protein A affinity chromatography

Storage: Store at -20° C.

Restrictions: For Research Use only

Publications

Product: 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).