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

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Title: High-dose biotin restores redox balance, energy and lipid homeostasis, and axonal health in a model of adrenoleukodystrophy.
Authors: Fourcade S; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain., Goicoechea L; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain., Parameswaran J; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain., Schlüter A; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain., Launay N; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain., Ruiz M; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain., Seyer A; Medday Pharmaceuticals SA, Paris, France., Colsch B; Service de Pharmacologie et Immuno-Analyse (SPI), Laboratoire d'Etude du Métabolisme des Médicaments, CEA, INRA, Université Paris Saclay, MetaboHUB, Gif-sur-Yvette, F-91191, France., Calingasan NY; Feil Family Brain and Mind Research Institute, Weill Cornell Medical College, New York, NY, 10065, USA., Ferrer I; Department of Pathology and Experimental Therapeutics, IDIBELL, Faculty of Medicine, University of Barcelona, L'Hospitalet de Llobregat, Barcelona, 08907, Spain.; Center for Biomedical Research on Neurodegenerative Diseases (CIBERNED), ISCIII, Madrid, Spain.; Institute of Neurosciences, University of Barcelona, Barcelona, Spain., Beal MF; Feil Family Brain and Mind Research Institute, Weill Cornell Medical College, New York, NY, 10065, USA., Sedel F; Medday Pharmaceuticals SA, Paris, France., Pujol A; Neurometabolic Diseases Laboratory, IDIBELL, Barcelona, Spain.; CIBERER U759, Center for Biomedical Research on Rare Diseases, Barcelona, Spain.; Catalan Institution of Research and Advanced Studies (ICREA), Barcelona, Spain.
Source: Brain pathology (Zurich, Switzerland) [Brain Pathol] 2020 Sep; Vol. 30 (5), pp. 945-963. Date of Electronic Publication: 2020 Jul 07.
Publication Type: Journal Article; Research Support, Non-U.S. Gov't
Journal Info: Publisher: International Society of Neuropathology Country of Publication: Switzerland NLM ID: 9216781 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1750-3639 (Electronic) Linking ISSN: 10156305 NLM ISO Abbreviation: Brain Pathol Subsets: MEDLINE
Database: MEDLINE Ultimate
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ISSN:1750-3639
DOI:10.1111/bpa.12869