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Journal articles
Potential of fibrates in the treatment of fatty acid oxidation disorders: Revival of classical drugs?
F. Djouadi
1, 2
F. Aubey
2
D. Schlemmer
2
S. Gobin
2
P. Laforet
3
R.J.A. Wanders
4
A.W. Strauss
5
J. Bonnefont
2
J. Bastin
2
J. Bastin 2
Author
1
CRC (UMR_S_1138 / U1138) - Centre de Recherche des Cordeliers (15 rue de l'Ecole de Médecine - Bâtiment B -
75270 Paris Cedex 06 - France)
- EPHE - École pratique des hautes études (4-14 Rue Ferrus, 75014 Paris - France)
- PSL - Université Paris sciences et lettres (60 rue Mazarine 75006 Paris - France)
- INSERM - Institut National de la Santé et de la Recherche Médicale : U1138 (101, rue de Tolbiac, 75013 Paris - France)
- SU - Sorbonne Université (21 rue de l’École de médecine - 75006 Paris - France)
- UP - Université de Paris : UMR_S_1138 (85 boulevard Saint-Germain 75006 Paris - France)
2
Inserm U393 - Handicaps génétiques de l'enfant (GH Necker 149, rue de Sèvres 75743 Paris cedex 15 - France)
- UPD5 - Université Paris Descartes - Paris 5 : UMR_S 393 (12, rue de l'École de Médecine - 75270 Paris cedex 06 - France)
- INSERM - Institut National de la Santé et de la Recherche Médicale : U393 (101, rue de Tolbiac, 75013 Paris - France)
3
CHU Pitié-Salpêtrière [AP-HP] (47-83 Boulevard de l'Hôpital, 75013 Paris - France)
- Sorbonne Université (France)
- AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP) (France)
4
AMC - Academic Medical Center - Academisch Medisch Centrum [Amsterdam] (Meibergdreef 9 1105 AZ Amsterdam - Netherlands)
- UvA - University of Amsterdam [Amsterdam] (Spui 21 1012 WX Amsterdam - Netherlands)
5
Vanderbilt University [Nashville] (Nashville, Tennessee, TN 37240 - United States)
Abstract : Exposure to fibrates leads to normalization of fatty acid oxidation (FAO) in fibroblasts from patients with myopathic forms of CPT2 deficiency or VLCAD deficiency. Correction of FAO is related to a drug-induced increase of residual enzyme activity, and this could provide a new treatment strategy for these disorders.
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Journal articles
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https://www.hal.inserm.fr/inserm-02896275
Contributor : Fatima Djouadi <>
Submitted on : Friday, July 10, 2020 - 1:54:05 PM
Last modification on : Wednesday, June 2, 2021 - 4:26:29 PM
Long-term archiving on: : Monday, November 30, 2020 - 8:04:53 PM
Contributor : Fatima Djouadi <>
Submitted on : Friday, July 10, 2020 - 1:54:05 PM
Last modification on : Wednesday, June 2, 2021 - 4:26:29 PM
Long-term archiving on: : Monday, November 30, 2020 - 8:04:53 PM
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Identifiers
- HAL Id : inserm-02896275, version 1
- DOI : 10.1007/s10545-006-0266-3
- PUBMED : 16763897
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INSERM | CORDELIERS | EPHE | UNIV-PARIS5 | UP-SANTE | SU-SCIENCES | SORBONNE-UNIVERSITE | USPC | UNIV-PARIS | PSL | SU-TI
Citation
F. Djouadi, F. Aubey, D. Schlemmer, S. Gobin, P. Laforet, et al.. Potential of fibrates in the treatment of fatty acid oxidation disorders: Revival of classical drugs?. Journal of Inherited Metabolic Disease, Springer Verlag, 2006, 29 (2-3), pp.341-342. ⟨10.1007/s10545-006-0266-3⟩. ⟨inserm-02896275⟩
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