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Title

Damage Mediated by Dysfunction of Organelles and Cellular Systems: Lysosomes

AuthorsMarí, Montserrat ; Fernández-Checa, José C.
KeywordsLysosomal membrane permeabilization
LSDs
LMP
Lipotoxicity
Gangliosides
Lysosomal storage disorders
Lysosome
Sphingolipids
Cathepsin
Cholesterol
Issue Date1-Aug-2014
PublisherElsevier
CitationPathobiology of Human Disease: A Dynamic Encylcopedia of Disease Mechanisms: 97-107 (2014)
AbstractLysosomes were discovered more than five decades ago and characterized as cytoplasmic organelles loaded with acid hydrolases responsible for the digestion of macromolecules and cellular debris. The membrane bilayer surrounding the lysosomes not only limits the nonspecific diffusion of the molecular digestive machinery to the cytosol but also ensures the acid pH required for the activity of acid hydrolases. This specialized digestive function is critical in the maintenance of cellular homeostasis and energy metabolism by clearing wasted constituents and damaged organelles. Besides these critical functions, lysosomes participate in apoptotic pathways due to the permeabilization of the lysosomal membrane and subsequent release of cathepsins, which recruit mitochondria to execute cell death, and are key regulators of autophagy. Alterations in lysosomal composition due to the accumulation of metabolites and lipids can have a broad impact in cellular homeostasis by modulating the fusion of autophagosomes with lysosomes for final cargo disposition. Accumulation of lipid intermediates in lysosomes is thought to contribute to the disruption in cellular homeostasis and metabolism, underlying the widespread pathological manifestations of many lysosomal storage diseases (LSDs). Thus, a better understanding of the biology of lysosomes may be of relevance in cellular physiology and in the treatment of LSDs
DescriptionCapítulo en: McManus, Linda M.; Mitchell, Richard N. (eds.). Pathobiology of Human Disease: A Dynamic Encylcopedia of Disease Mechanisms. Amsterdam: Elsevier, 2014, p.97-107. ISBN 978-0-12-386457-4
Publisher version (URL)http://dx.doi.org/10.1016/B978-0-12-386456-7.01406-4
URIhttp://hdl.handle.net/10261/128473
DOI10.1016/B978-0-12-386456-7.01406-4
Identifiersdoi: 10.1016/B978-0-12-386456-7.01406-4
isbn: 978-0-12-386457-4
Appears in Collections:(IIBB) Libros y partes de libros
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