23 products were found matching "K01052"!

The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Human LAL. Standards or samples are added to the appropriate microtiter plate wells then with a biotin-conjugated antibody specific to Human LAL. Next,...

The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Mouse LIPA. Standards or samples are added to the appropriate microtiter plate wells then with a biotin-conjugated antibody specific to Mouse LIPA. Next,...

Cholesterol ester storage disease and Wolman disease are recessive autosomal disorders caused by a deficiency in lysosomal acid lipase (LAL), also known as cholesteryl ester hydrolase. 4-Methylumbelliferyl palmitate (4-MUP) is a fluorogenic substrate for lysosomal acid lypase (LAL). 4-MUP is cleaved by LAL to...

Lalistat 2 is an inhibitor of lysosomal acid lipase (LAL, IC50 = 152 nM using purified human LAL). It blocks lipid clearance induced by the autophagy enhancers MSL and MSL-7 in HeLa cells preloaded with palmitic acid (Cay-10006627) and oleic acid (Cay-90260).Formal Name: 1-piperidinecarboxylic acid,...

4-Methylumbelliferyl (4-MU) oleate is a fluorogenic substrate for acid and alkaline lipases. It is cleaved by lipases, liberating 4-MU, which has an emission maximum at 445-454 nm. The excitation maximum for 4-MU is pH-dependent: 330, 370, and 385 nm at pH 4.6, 7.4, and 10.4, respectively.Formal Name:...

Lalistat 1 is an inhibitor of lysosomal acid lipase (LAL, IC50 = 68 nM using purified human LAL). It is selective for LAL over human pancreatic and bovine milk lipoprotein lipases up to a concentration of 10 µM. Lalistat 1 (0.01-10 µM) reduces cholesterol accumulation in lysosome-like storage organelles in GM03123...

This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced...

Lysosomal acid lipase (LAL) is a lysosomal enzyme that hydrolyzes cholesteryl esters and triglycerides to produce cholesterol, glycerol, and free fatty acids. LAL deficiency is due to mutations in the LAL gene, LIPA, that lead to decreases in LAL activity. Wolman's disease is a severe form of LAL deficiency that...

This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced...

This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced...

Protein function: Catalyzes the deacylation of triacylglyceryl and cholesteryl ester core lipids of endocytosed low density lipoproteins to generate free fatty acids and cholesterol. [The UniProt Consortium] Buffer: 40% glycerol and PBS (pH 7.2). 0.02% sodium azide is added as preservative. Highest antigen sequence...