Drug repositioning in neurodegeneration: An overview of the use of ambroxol in neurodegenerative diseases.
Ambroxol
/ pharmacology
Amyotrophic Lateral Sclerosis
/ drug therapy
Animals
Disease Models, Animal
Disease Progression
Drug Repositioning
Enzyme Inhibitors
/ pharmacology
Glucosylceramidase
/ antagonists & inhibitors
Humans
Mutation
Nerve Degeneration
Neuroprotective Agents
/ pharmacology
Spinal Cord
/ drug effects
Superoxide Dismutase-1
/ genetics
beta-Glucosidase
/ antagonists & inhibitors
ALS
Ambroxol
GBA2
Glucosylceramide
Neurodegenerative disease
Journal
European journal of pharmacology
ISSN: 1879-0712
Titre abrégé: Eur J Pharmacol
Pays: Netherlands
ID NLM: 1254354
Informations de publication
Date de publication:
05 Oct 2020
05 Oct 2020
Historique:
received:
14
04
2020
revised:
30
06
2020
accepted:
29
07
2020
pubmed:
3
8
2020
medline:
19
5
2021
entrez:
3
8
2020
Statut:
ppublish
Résumé
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease in adults. While it is primarily characterized by the death of upper and lower motor neurons, there is a significant metabolic component involved in the progression of the disease. Two-thirds of ALS patients have metabolic alterations that are associated with the severity of symptoms. In ALS, as in other neurodegenerative diseases, the metabolism of glycosphingolipids, a class of complex lipids, is strongly dysregulated. We therefore assume that this pathway constitutes an interesting avenue for therapeutic approaches. We have shown that the glucosylceramide degrading enzyme, glucocerebrosidase (GBA) 2 is abnormally increased in the spinal cord of the SOD1
Identifiants
pubmed: 32739173
pii: S0014-2999(20)30538-0
doi: 10.1016/j.ejphar.2020.173446
pii:
doi:
Substances chimiques
Enzyme Inhibitors
0
Neuroprotective Agents
0
Ambroxol
200168S0CL
Sod1 protein, mouse
EC 1.15.1.1
Superoxide Dismutase-1
EC 1.15.1.1
beta-Glucosidase
EC 3.2.1.21
beta-glucosidase 2, mouse
EC 3.2.1.21
GBA2 protein, human
EC 3.2.1.45
Glucosylceramidase
EC 3.2.1.45
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
173446Informations de copyright
Copyright © 2020. Published by Elsevier B.V.