Ceramide, sphingomyelin, and glycosphingolipids (both neutral and acidic) are characterized by the presence in the lipid moiety of an aliphatic base known as sphingosine. Altogether, they are called sphingolipids and are particularly abundant in neuronal plasma membranes, where, via interactions with the other membrane lipids and membrane proteins, they play a specific role in modulating the cell signaling processes. The metabolic pathways determining the plasma membrane sphingolipid composition are thus the key point for functional changes of the cell properties. Unnatural changes of the neuronal properties are observed in sphingolipidoses, lysosomal storage diseases occurring when a lysosomal sphingolipid hydrolase is not working, leading to the accumulation of the substrate and to its distribution to all the cell membranes interacting with lysosomes. Moreover, secondary accumulation of sphingolipids is a common trait of other lysosomal storage diseases.

Sphingolipids and neuronal degeneration in lysosomal storage disorders / S. Grassi, E. Chiricozzi, L. Mauri, S. Sonnino, A. Prinetti. - In: JOURNAL OF NEUROCHEMISTRY. - ISSN 0022-3042. - (2018 Jun 30). [Epub ahead of print] [10.1111/jnc.14540]

Sphingolipids and neuronal degeneration in lysosomal storage disorders

S. Grassi
Primo
;
E. Chiricozzi
Secondo
;
L. Mauri;S. Sonnino
Penultimo
;
A. Prinetti
Ultimo
2018

Abstract

Ceramide, sphingomyelin, and glycosphingolipids (both neutral and acidic) are characterized by the presence in the lipid moiety of an aliphatic base known as sphingosine. Altogether, they are called sphingolipids and are particularly abundant in neuronal plasma membranes, where, via interactions with the other membrane lipids and membrane proteins, they play a specific role in modulating the cell signaling processes. The metabolic pathways determining the plasma membrane sphingolipid composition are thus the key point for functional changes of the cell properties. Unnatural changes of the neuronal properties are observed in sphingolipidoses, lysosomal storage diseases occurring when a lysosomal sphingolipid hydrolase is not working, leading to the accumulation of the substrate and to its distribution to all the cell membranes interacting with lysosomes. Moreover, secondary accumulation of sphingolipids is a common trait of other lysosomal storage diseases.
gangliosides; lysosome; membrane fusion; sphingolipidoses; sphingolipids; sphingomyelin
Settore BIO/10 - Biochimica
30-giu-2018
30-giu-2018
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/580444
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