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: Mouse

Reactivity: Human

Application: Western Blotting (WB), ELISA

Certificates: ISO 9001:2008

Catalog no.: ABIN659253

Additional Information

Alternative name: LAL

Gene ID: 3988

UniProt: P38571, NP_001121077.1

Immunogen: Purified recombinant fragment of LAL expressed in E. Coli.

Isotype: IgG2a

Description: Other names: LAL, CESD, LIPA

Characteristics: Mouse Monoclonal Antibody to LAL

Specificity: Purified recombinant fragment of LAL expressed in E. Coli.

Molecular Weight: 45419 DA

Comments:

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 Details

Application Notes: The suggested dilution is: ELISA: ~~ 1:10000. Western Bloting: ~~ 1:500 - 2000.

Buffer: Purified recombinant fragment of LAL expressed in E. Coli.

Storage: LAL Antibody can be stored at 4?, for long term storage, store at -20?.

Research Area: Metabolism, Cell Structure

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).