LIPA
Reactivité: Humain, Rat, Cheval, Porc, Boeuf (Vache), Lapin, Mouton
WB
Hôte: Lapin
Polyclonal
unconjugated
Indications d'application
Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Buffer
PBS, 0,03 % sodium azide
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Antigène
Lipase A (LIPA)
(Lipase A, Lysosomal Acid, Cholesterol Esterase (LIPA))
Autre désignation
LIPA
Sujet
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.Synonyms: Acid cholesteryl ester hydrolase, Cholesteryl esterase, EC=3.1.1.13, LAL, LIPA, Lipase A, Lysosomal acid lipase/cholesteryl ester hydrolase, Sterol esterase