Cayman's LAL Activity MaxSpec® Assay Kit includes the necessary reagents to quantify lysosomal acid lipase (LAL) activity in dried blood spots. The LAL Activity MaxSpec® Assay Substrate (P-PMHC) has been designed to function as a highly selective substrate for LAL

Lysosomal Acid Lipase Activity MaxSpec® Assay Kit – 1 ea

Brand:
Cayman
Storage:
-20

Lysosomal acid lipase (LAL) is a lysosomal enzyme that hydrolyzes cholesteryl esters and triglycerides to produce cholesterol, glycerol, and free fatty acids.{42857} 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 begins in infancy and is characterized by a nearly complete or complete lack of LAL activity resulting in gastrointestinal disorders, hepatomegaly, and failure to thrive, leading to hypercholesterolemia and fatality within months without treatment. Cholesterol ester storage disorder is a less severe form of LAL deficiency in which LAL activity is reduced but not abolished. It presents later in life and is characterized by gastrointestinal disturbances, dyslipidemia, hepatomegaly, and impaired liver function. Research towards development of methods to detect deficiency in this enzyme has become an important goal in diagnosing and treating individuals with this disorder.  

 

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SKU: 24854 - 1 ea Category:

Description

Cayman’s LAL Activity MaxSpec® Assay Kit includes the necessary reagents to quantify lysosomal acid lipase (LAL) activity in dried blood spots. The LAL Activity MaxSpec® Assay Substrate (P-PMHC) has been designed to function as a highly selective substrate for LAL, which hydrolyzes the fatty acyl group of P-PMHC resulting in the formation of the LAL Activity MaxSpec® Assay Product 4-propyl-8-methyl-7-hydroxycoumarin (PMHC). Using the LAL Activity MaxSpec® Assay Kit, LAL activity may be quantified directly by fluorometry or by LC-MS in combination with the LAL Activity MaxSpec® Assay Internal Standard. ,

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Synonyms:  LAL

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Application|Enzyme Activity||Application|Fluorescence||Application|Mass Spectrometry||Product Type|Assay Kits|MaxSpec® Kits||Research Area|Endocrinology & Metabolism|Metabolic Diseases|Dyslipidemias||Research Area|Lipid Biochemistry|Fatty Acids||Research Area|Lipid Biochemistry|Glycerolipids

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