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Lysosomal acid lipase/cholesteryl ester hydrolase

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Crucial for the intracellular hydrolysis of cholesteryl esters and triglycerides that have been internalized via receptor-mediated endocytosis of lipoprotein particles. Important in mediating the effect of LDL (low density lipoprotein) uptake on suppression of hydroxymethylglutaryl-CoA reductase and activation of endogenous cellular cholesteryl ester formation.

Below are the list of possible Lysosomal acid lipase/cholesteryl ester hydrolase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Lysosomal acid lipase/cholesteryl ester hydrolase

 Lysosomal acid lipase/cholesteryl ester hydrolase ELISA Kit
 Lysosomal acid lipase/cholesteryl ester hydrolase Recombinant
 Lysosomal acid lipase/cholesteryl ester hydrolase Antibody
Also known as Lysosomal acid lipase/cholesteryl ester hydrolase (Acid cholesteryl ester hydrolase) (LAL) (Cholesteryl esterase) (Lipase A) (Sterol esterase).
LIPA: Crucial for the intracellular hydrolysis of cholesteryl esters and triglycerides that have been internalized via receptor- mediated endocytosis of lipoprotein particles. Important in mediating the effect of LDL (low density lipoprote
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in) uptake on suppression of hydroxymethylglutaryl-CoA reductase and activation of endogenous cellular cholesteryl ester formation. Defects in LIPA are the cause of Wolman disease (WOD). WOD is a severe manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. WOD occurs in infancy and is nearly always fatal before the age of 1 year. Defects in LIPA are the cause of cholesteryl ester storage disease (CESD). CESD is a mild manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. It is characterized by late-onset. Belongs to the AB hydrolase superfamily. Lipase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.1.1.13; Hydrolase; Lipid Metabolism - steroid biosynthesis

Chromosomal Location of Human Ortholog: 10q23.2-q23.3

Molecular Function: lipase activity; sterol esterase activity

Disease: Lysosomal Acid Lipase Deficiency
 LIPA ELISA Kit
 LIPA Recombinant
 LIPA Antibody
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