caa a22 4500 467933006 CHVBK 20180406152948.0 cr unu---uuuuu 170328e20060401xx s 000 0 eng 10.1007/s10545-006-0266-3 doi (NATIONALLICENCE)springer-10.1007/s10545-006-0266-3 Potential of fibrates in the treatment of fatty acid oxidation disorders: Revival of classical drugs? [Elektronische Daten] [F. Djouadi, F. Aubey, D. Schlemmer, S. Gobin, P. Laforet, R. Wanders, A. Strauss, J. Bonnefont, J. Bastin] Summary: 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. SSIEM and Springer, 2006 Djouadi F. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut Aubey F. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut Schlemmer D. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut Gobin S. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut Laforet P. Institut de Myologie, Hôpital La Pitié-Salpétrière, Paris, France aut Wanders R. Laboratory for Genetic Metabolic Diseases, Academic Medical Centre, Amsterdam, The Netherlands aut Strauss A. Vanderbilt Children's Hospital, Nashville, Tennessee, USA aut Bonnefont J. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut Bastin J. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/2-3(2006-04-01), 341-342 0141-8955 29:2-3<341 2006 29 10545 https://doi.org/10.1007/s10545-006-0266-3 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0266-3 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Djouadi F. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut NATIONALLICENCE 700 1- Aubey F. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut NATIONALLICENCE 700 1- Schlemmer D. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut NATIONALLICENCE 700 1- Gobin S. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut NATIONALLICENCE 700 1- Laforet P. Institut de Myologie, Hôpital La Pitié-Salpétrière, Paris, France aut NATIONALLICENCE 700 1- Wanders R. Laboratory for Genetic Metabolic Diseases, Academic Medical Centre, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Strauss A. Vanderbilt Children's Hospital, Nashville, Tennessee, USA aut NATIONALLICENCE 700 1- Bonnefont J. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut NATIONALLICENCE 700 1- Bastin J. INSERM U393, Hôpital Necker, Tour Lavoisier, 149 rue de Sèvres, 75015, Paris, France aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/2-3(2006-04-01), 341-342 0141-8955 29:2-3<341 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer