Lysosomal acid lipase (LIPA) Mouse Monoclonal Antibody [Clone ID: 9G7F12]

Specifications

Product Data
Clone Name	9G7F12
Applications	ELISA, WB
Recommend Dilution	Western Blot: 1/500 - 1/2000. ELISA: 1/10000.
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Purified recombinant fragment of LAL expressed in E. Coli.
Specificity	This antibody reacts to LAL.
Formulation	PBS State: Purified State: Liquid purified Ig fraction Preservative: 0,03% sodium azide
Conjugation	Unconjugated
Storage Condition	Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	lipase A, lysosomal acid type
Database Link	Entrez Gene 3988 Human UniProt ID P38571
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.
Synonyms	LIPA, Lysosomal acid lipase/cholesteryl ester hydrolase, LAL, Acid cholesteryl ester hydrolase, EC=3.1.1.13, Sterol esterase, Lipase A, Cholesteryl esterase
Reference Data

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