LysoLive™ Lysosomal Acid Lipase Assay Kit
(ab253380)
Key features and details
- Detection method: Fluorescent
Overview
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Product name
LysoLive™ Lysosomal Acid Lipase Assay Kit
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Detection method
Fluorescent -
Product overview
This kit utilizes the sensitive substrate LipaGreen™ to monitor acid lipase activity in the lysosomes of live cells by flow cytometry.
The LysoLive™ Lysosomal Acid Lipase Assay Kit utilizes a sensitive substrate for lysosomal acid lipase (LAL), an enzyme indicated in several diseases such as Lysosomal Acid Lipase Deficiency (LALD). The substrate only becomes fluorescent upon cleavage by the enzyme meaning lysosomes with low acid lipase activity will have reduced staining in a manner proportional to the enzyme activity levels found in the cell.
This kit is sold by Marker Gene under product code M2887.
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Notes
Important Licensing Information: This product may be covered by one or more United States or International Patents and is subject to our Limited Use Label Licenses. By use of this product, you accept the terms and conditions of all applicable Limited Use Label Licenses.
Properties
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Storage instructions
Please refer to protocols. -
Components 1 units DMSO 1 x 500µl LysoLive™ LipaGreen 5 units MarkerGene™ Flow Holding and Sorting Buffer for Adherent Cells 1 x 10ml MarkerGene™ Flow Holding and Sorting Buffer for non-Adherent Cells 1 x 10ml -
Research areas
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Function
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. -
Involvement in disease
Defects in LIPA are the cause of Wolman disease (WOD) [MIM:278000]. 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) [MIM:278000]. 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. -
Sequence similarities
Belongs to the AB hydrolase superfamily. Lipase family. -
Cellular localization
Lysosome. - Information by UniProt
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Alternative names
- Acid cholesteryl ester hydrolase
- CESD
- cholesterol ester hydrolase
see all
Images
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Labeling cells with LysoLive Lysosomal Acid Lipase Assay Kit in healthy cells and those from a LALD patient.
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Labeling of healthy cells with LysoLive Lysosomal Assay Kit after treatment with Lipase inhibiter (Orlistat).
