High-dose biotin restores redox balance, energy and lipid homeostasis, and axonal health in a model of adrenoleukodystrophy.
ATP Binding Cassette Transporter, Subfamily D, Member 1
/ genetics
Adrenoleukodystrophy
/ genetics
Animals
Axons
/ metabolism
Biotin
/ metabolism
Cell Line
Disease Models, Animal
Energy Metabolism
Homeostasis
Humans
Lipids
Mechanistic Target of Rapamycin Complex 1
/ metabolism
Mice
Mice, Inbred C57BL
Mice, Knockout
Oxidation-Reduction
/ drug effects
Oxidative Stress
/ physiology
Sterol Regulatory Element Binding Protein 1
/ metabolism
NRF2
SREBP-1c
axonal degeneration
biotin
mTORC1
multiple sclerosis
redox homeostasis
Journal
Brain pathology (Zurich, Switzerland)
ISSN: 1750-3639
Titre abrégé: Brain Pathol
Pays: Switzerland
ID NLM: 9216781
Informations de publication
Date de publication:
09 2020
09 2020
Historique:
received:
28
02
2020
revised:
24
05
2020
accepted:
26
05
2020
pubmed:
9
6
2020
medline:
17
8
2021
entrez:
9
6
2020
Statut:
ppublish
Résumé
Biotin is an essential cofactor for carboxylases that regulates the energy metabolism. Recently, high-dose pharmaceutical-grade biotin (MD1003) was shown to improve clinical parameters in a subset of patients with chronic progressive multiple sclerosis. To gain insight into the mechanisms of action, we investigated the efficacy of high-dose biotin in a genetic model of chronic axonopathy caused by oxidative damage and bioenergetic failure, the Abcd1
Identifiants
pubmed: 32511826
doi: 10.1111/bpa.12869
pmc: PMC8018149
doi:
Substances chimiques
ATP Binding Cassette Transporter, Subfamily D, Member 1
0
Abcd1 protein, mouse
0
Lipids
0
Srebf1 protein, mouse
0
Sterol Regulatory Element Binding Protein 1
0
Biotin
6SO6U10H04
Mechanistic Target of Rapamycin Complex 1
EC 2.7.11.1
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
945-963Subventions
Organisme : CIBERER
Pays : International
Organisme : Instituto de Salud Carlos III
ID : CPII16/00016
Pays : International
Organisme : Instituto de Salud Carlos III
ID : PI17/00916
Pays : International
Organisme : Agència de Gestió d'Ajuts Universitaris i de Recerca
ID : 2017SGR1206
Pays : International
Organisme : Agència de Gestió d'Ajuts Universitaris i de Recerca
ID : SGR 2014SGR1430
Pays : International
Organisme : European Regional Development Fund
Pays : International
Organisme : European Social Fund
Pays : International
Informations de copyright
© 2020 International Society of Neuropathology.
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