High-dose biotin restores redox balance, energy and lipid homeostasis, and axonal health in a model of adrenoleukodystrophy.

Fourcade, Stéphane; Goicoechea, Leire; Parameswaran, Janani; Schlüter, Agatha; Launay, Nathalie; Ruiz, Montserrat; Seyer, Alexandre; Colsch, Benoit; Calingasan, Noel Ylagan; Ferrer, Isidre; Beal, M Flint; Sedel, Frédéric; Pujol, Aurora

Fourcade, Stéphane; Goicoechea, Leire; Parameswaran, Janani; Schlüter, Agatha; Launay, Nathalie; Ruiz, Montserrat; Seyer, Alexandre; Colsch, Benoit; Calingasan, Noel Ylagan; Ferrer, Isidre; Beal, M Flint; Sedel, Frédéric; Pujol, Aurora.

Affiliation

Fourcade S; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.
Goicoechea L; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.
Parameswaran J; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.
Schlüter A; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.
Launay N; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.
Ruiz M; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.
Seyer A; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.
Colsch B; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.
Calingasan NY; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.
Ferrer I; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.
Beal MF; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.
Sedel F; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.
Pujol A; Medday Pharmaceuticals SA, Paris, France.

Brain Pathol ; 30(5): 945-963, 2020 09.

Article in En | MEDLINE | ID: mdl-32511826

Subject(s)

Adrenoleukodystrophy/therapy; Biotin/pharmacology; ATP Binding Cassette Transporter, Subfamily D, Member 1/genetics; ATP Binding Cassette Transporter, Subfamily D, Member 1/metabolism; Adrenoleukodystrophy/genetics; Adrenoleukodystrophy/metabolism; 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

Key words

NRF2; SREBP-1c; axonal degeneration; biotin; mTORC1; multiple sclerosis; redox homeostasis

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Full text: 1 Database: MEDLINE Main subject: Biotin / Adrenoleukodystrophy Type of study: Prognostic_studies Language: En Journal: Brain Pathol Year: 2020 Type: Article Affiliation country: Spain

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