Ambroxol Hydrochloride Improves Motor Functions and Extends Survival in a Mouse Model of Familial Amyotrophic Lateral Sclerosis.
ALS
GBA2
ambroxol
glucocerebrosidase
glucosylceramide
neuromuscular junction
Journal
Frontiers in pharmacology
ISSN: 1663-9812
Titre abrégé: Front Pharmacol
Pays: Switzerland
ID NLM: 101548923
Informations de publication
Date de publication:
2019
2019
Historique:
received:
21
02
2019
accepted:
15
07
2019
entrez:
27
8
2019
pubmed:
27
8
2019
medline:
27
8
2019
Statut:
epublish
Résumé
Amyotrophic lateral sclerosis (ALS) is a multifactorial and fatal neurodegenerative disease. Growing evidence connects sphingolipid metabolism to the pathophysiology of ALS. In particular, levels of ceramides, glucosylceramides, and gangliosides are dysregulated in the central nervous system and at the neuromuscular junctions of both animal models and patients. Glucosylceramide is the main precursor of complex glycosphingolipids that is degraded by lysosomal (GBA1) or non-lysosomal (GBA2) glucocerebrosidase. Here, we report that GBA2, but not GBA1, activity is markedly increased in the spinal cord, of SOD1
Identifiants
pubmed: 31447678
doi: 10.3389/fphar.2019.00883
pmc: PMC6692493
doi:
Types de publication
Journal Article
Langues
eng
Pagination
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