Lyso-glycosphingolipids: presence and consequences.

Acid Ceramidase / metabolism Animals Enzyme Replacement Therapy / methods Genetic Therapy / methods Glycosphingolipids / biosynthesis Humans Immunity Lysosomal Storage Diseases / drug therapy Lysosomes / metabolism Molecular Targeted Therapy / methods
acid ceramidase glycosphingolipid immune response lysosomal storage disease

Journal

Essays in biochemistry
ISSN: 1744-1358
Titre abrégé: Essays Biochem
Pays: England
ID NLM: 0043306

Informations de publication

Date de publication:
23 09 2020
Historique:
received: 05 05 2020
revised: 14 07 2020
accepted: 17 07 2020
pubmed: 19 8 2020
medline: 9 2 2021
entrez: 19 8 2020
Statut: ppublish

Résumé

Lyso-glycosphingolipids are generated in excess in glycosphingolipid storage disorders. In the course of these pathologies glycosylated sphingolipid species accumulate within lysosomes due to flaws in the respective lipid degrading machinery. Deacylation of accumulating glycosphingolipids drives the formation of lyso-glycosphingolipids. In lysosomal storage diseases such as Gaucher Disease, Fabry Disease, Krabbe disease, GM1 -and GM2 gangliosidosis, Niemann Pick type C and Metachromatic leukodystrophy massive intra-lysosomal glycosphingolipid accumulation occurs. The lysosomal enzyme acid ceramidase generates the deacylated lyso-glycosphingolipid species. This review discusses how the various lyso-glycosphingolipids are synthesized, how they may contribute to abnormal immunity in glycosphingolipid storing lysosomal diseases and what therapeutic opportunities exist.

Identifiants

DOI: 10.1042/EBC20190090 PMID: 32808655 PMC: PMC7517347
pubmed: 32808655
pii: 226135
doi: 10.1042/EBC20190090
pmc: PMC7517347
doi:

Substances chimiques

Glycosphingolipids 0
Acid Ceramidase EC 3.5.1.23

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

565-578

Informations de copyright

© 2020 The Author(s).

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Auteurs

Marco van Eijk (M)

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

Maria J Ferraz (MJ)

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

Rolf G Boot (RG)

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

Johannes M F G Aerts (JMFG)

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

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Classifications MeSH

questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Hydrolases › Amidohydrolases › Ceramidases › Acid Ceramidase › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Eucaryotes › Animaux › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Thérapeutique › Traitement médicamenteux › Thérapie enzymatique › Thérapie enzymatique substitutive › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Techniques d'investigation › Techniques génétiques › Génie génétique › Thérapie génétique › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Lipides › Lipides membranaires › Sphingolipides › Glycosphingolipides › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Phénomènes du système immunitaire › Immunité › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Maladies lysosomiales › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Cellules › Structures cellulaires › Espace intracellulaire › Cytoplasme › Structures cytoplasmiques › Organites › Vésicules cytoplasmiques › Lysosomes › Lyso-glycosphingolipids: presence and consequences.
questionsmedicales.fr › Thérapeutique › Traitement médicamenteux › Thérapie moléculaire ciblée › Lyso-glycosphingolipids: presence and consequences.