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Alternatively, fatty acids can be converted to lipid intermediates like diacylglycerol, ceramides and fatty acyl-CoAs. These lipid intermediates can impair cellular function, which is referred to as lipotoxicity. [2] Adipocytes, the cells that normally function as lipid store of the body, are well equipped to handle the excess lipids. Yet, too ...
No single mechanism leading to steatosis exists; rather, a varied multitude of pathologies disrupt normal lipid movement through the cell and cause accumulation. [7] These mechanisms can be separated based on whether they ultimately cause an oversupply of lipid which can not be removed quickly enough (i.e., too much in), or whether they cause a failure in lipid breakdown (i.e., not enough used).
Since lipids are hydrophobic molecules, they need to be solubilized before their metabolism can begin. Lipid metabolism often begins with hydrolysis, [7] which occurs with the help of various enzymes in the digestive system. [2] Lipid metabolism also occurs in plants, though the processes differ in some ways when compared to animals. [8]
Sphingolipidoses are a class of lipid storage disorders or degenerative storage disorders caused by deficiency of an enzyme that is required for the catabolism of lipids that contain ceramide, [1] also relating to sphingolipid metabolism.
Also, glycogen storage disease type II (Pompe disease) is a defect in lysosomal metabolism as well, [6] although it is otherwise classified into E74.0 in ICD-10. Cystinosis is an lysosomal storage disease characterized by the abnormal accumulation of the amino acid cystine. [citation needed]
Examples of various lipid species. Lipidomics is the large-scale study of pathways and networks of cellular lipids in biological systems. [1] [2] [3] The word "lipidome" is used to describe the complete lipid profile within a cell, tissue, organism, or ecosystem and is a subset of the "metabolome" which also includes other major classes of biological molecules (such as amino acids, sugars ...
The surface of a curved lipid bilayer. Lipidology is the scientific study of lipids. Lipids are a group of biological macromolecules that have a multitude of functions in the body. [1] [2] [3] Clinical studies on lipid metabolism in the body have led to developments in therapeutic lipidology for disorders such as cardiovascular disease. [4]
Gaucher's disease is the most common of the lysosomal storage diseases. [2] It is a form of sphingolipidosis (a subgroup of lysosomal storage diseases), as it involves dysfunctional metabolism of sphingolipids. [3] The disease is named after the French physician Philippe Gaucher, who originally described it in 1882. [4]