DJOUADI Fatima et BASTIN Jean : UMR_S1124 : Inborn mitochondrial disorders : pharmacological therapy and metabolic signaling

Unit : INSERM UMR_S1124 - Université Paris Descartes

Faculté des Sciences Fondamentales et Biomédicales
INSERM UMR_S 1124 Toxicologie, Pharmacologie et Signalisation Cellulaire
45 rue des Saints Pères
75270 Paris cedex 06

Speciality : Department DGNRV

Director of the unit : Robert BAROUKI

Principal investigator :

Email : Phone number: +33 (0)1 42 86 22 19

Composition of research team :


5 Recent publications of the research team :

Aires V, Delmas D, Le Bachelier C, Latruffe N, Schlemmer D, Benoist JF, Djouadi F and Bastin J.
Stilbenes and resveratrol metabolites improve fatty acid oxidation defects in human fibroblasts.
Orphanet J Rare Dis. 2014, 9 :79 

Lopes Costa A, Le Bachelier C, Mathieu L, Rotig A, Boneh A, De Lonlay P, Tarnopolsky MA, Thorburn DR, Bastin J and Djouadi F.
Beneficial effects of Resveratrol on respiratory chain defects in patients’ fibroblasts involve estrogen receptor and estrogen-related receptor signaling.
Hum. Mol. Genet. 2014, 23:2106-2119

Bastin J, Lopes-Costa A and Djouadi F.
Exposure to resveratrol triggers pharmacological correction of fatty acid utilization in human fatty acid oxidation-deficient fibroblasts.
Hum. Mol Genet. 2011, 20: 2048-2057

Bonnefont JP*, Bastin J* (*contribution égale), Laforêt P, Aubey F, Mogenet A, Romano S, Ricquier D, Gobin-Limballe S, Vassault A, Behin A, Eymard B, Bresson JL and Djouadi F.
Long term follow-up of bezafibrate treatment in the myopathic form of Carnitine-PalmitoylTransferase 2 deficiency
Clin Pharmacol Ther, 2010, 88: 101-108.

Gobin-Limballe S, McAndrew R, Djouadi F, Kim JJ and Bastin J.
Compared effects of missense mutations in Very Long Chain Acyl-CoA Dehydrogenase deficiency: combined analysis by structural, functional and pharmacological approaches.
BBA-Molecular Basis of Disease, 2010, 1802:478-84.