Glycosphingolipids and lysosomal storage disorders as illustrated by gaucher disease.

Animals Biomarkers / blood Gaucher Disease / diagnosis Glycosphingolipids / metabolism Humans
Gaucher disease Glucocerebrosidase Glucosylsphingosine Glycosphingolipids Lysosome

Journal

Current opinion in chemical biology
ISSN: 1879-0402
Titre abrégé: Curr Opin Chem Biol
Pays: England
ID NLM: 9811312

Informations de publication

Date de publication:
12 2019
Historique:
received: 12 07 2019
revised: 02 10 2019
accepted: 24 10 2019
pubmed: 30 11 2019
medline: 8 7 2020
entrez: 30 11 2019
Statut: ppublish

Résumé

Glycosphingolipids are important building blocks of the outer leaflet of the cell membrane. They are continuously recycled, involving fragmentation inside lysosomes by glycosidases. Inherited defects in degradation cause lysosomal glycosphingolipid storage disorders. The relatively common glycosphingolipidosis Gaucher disease is highlighted here to discuss new insights in the molecular basis and pathophysiology of glycosphingolipidoses reached by fundamental research increasingly using chemical biology tools. We discuss improvements in the detection of glycosphingolipid metabolites by mass spectrometry and review new developments in laboratory diagnosis and disease monitoring as well as therapeutic interventions.

Identifiants

DOI: 10.1016/j.cbpa.2019.10.006 PMID: 31783225
pubmed: 31783225
pii: S1367-5931(19)30086-9
doi: 10.1016/j.cbpa.2019.10.006
pii:
doi:

Substances chimiques

Biomarkers 0
Glycosphingolipids 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

204-215

Informations de copyright

Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.

Auteurs

Johannes M F G Aerts (JMFG)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands. Electronic address: j.m.f.g.aerts@lic.leidenuniv.nl.

Chi-Lin Kuo (CL)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands.

Lindsey T Lelieveld (LT)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands.

Daphne E C Boer (DEC)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands.

Martijn J C van der Lienden (MJC)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands.

Herman S Overkleeft (HS)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands.

Marta Artola (M)

Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, the Netherlands.

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Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › Glycosphingolipids and lysosomal storage...
questionsmedicales.fr › Facteurs biologiques › Marqueurs biologiques › Glycosphingolipids and lysosomal storage...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Maladies lysosomiales › Maladies neurologiques de surcharge lysosomiale › Sphingolipidoses › Maladie de Gaucher › Glycosphingolipids and lysosomal storage...
questionsmedicales.fr › Lipides › Lipides membranaires › Sphingolipides › Glycosphingolipides › Glycosphingolipids and lysosomal storage...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Glycosphingolipids and lysosomal storage...