AL-antilichaam (9G7F12]

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.

3988

P38571

P38571

Human

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

9G7F12

Unconjugated

Primary Antibodies

Homeostasis

Antibody is purified by protein A affinity chromatography.

Batch dependent

Purified antibody in PBS containing 0.03% sodium azide.

None

Blue Ice

LAL monoclonal antibody can be stored at -20˚ C, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

IgG2a

LIPA

Lipase A, lysosomal acid, cholesterol esterase

Homo sapiens

Uta Drebber, Matthias Andersen, Hans U Kasper, et al, World J Gastroenterol. 2005 Apr 21;11 (15) :2364-6.

Renata Boldrini, Rita Devito, R.Biselli, et al, Pathol Res Pract. 2004;200 (3) :231-40.

Cholesteryl esterase, Acid cholesteryl ester hydrolase, LAL, CESD

ELISA, WB

68067636