Description
Key facts
Detection method:Fluorescent
Product details:
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.
Properties and Storage Information:
Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-Multi, Appropriate long-term storage conditions-Multi, Storage information-Please refer to protocols
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Lysosomal acid lipase (LAL) also called lipase A is an essential enzyme with a mass of approximately 43 kDa. It is expressed within the lysosomes of many cell types and catalyzes the hydrolysis of cholesteryl esters and triglycerides into free cholesterol and free fatty acids. This process is important for the cellular lipid metabolism which provides essential signaling molecules and membrane components. The enzyme's activity varies across different tissue types with high expression levels observed in organs like the liver and the adrenal glands.
Biological function summary
Lysosomal acid lipase contributes to the breakdown and regulation of lipid storage within the cell. It is not known to be part of a larger protein complex but its function is critical for maintaining lipid homeostasis in the cell. Through its actions LAL supplies cholesterol and fatty acids essential for various cellular processes including membrane formation and energy production. Additionally it assists in cellular storage management by preventing the excessive accumulation of lipids.
Pathways
The enzymatic actions of lysosomal acid lipase are central to the cholesterol ester and triglyceride metabolic pathways. It plays an important role in the lysosome as part of the lipid degradation process impacting cholesterol transport pathways and maintaining lipid equilibrium. Its activities are closely related to those of other enzymes like hormone-sensitive lipase which also participate in lipid metabolism though within distinct compartments and contexts.
Lysosomal acid lipase is linked to lipid storage disorders such as Wolman disease and Cholesteryl Ester Storage Disease (CESD). These conditions result from LAL deficiency leading to harmful accumulation of cholesteryl esters and triglycerides in tissues. Through disease pathways LAL becomes interconnected with proteins affected by lipid dysregulation such as low-density lipoprotein receptors which play a part in maintaining lipid balance and can contribute to more severe cardiovascular conditions when impaired by LAL deficiency.
