Literature DB >> 16763897

Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs?

F Djouadi1, F Aubey, D Schlemmer, S Gobin, P Laforet, R J A Wanders, A W Strauss, J P Bonnefont, J Bastin.   

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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Year:  2006        PMID: 16763897     DOI: 10.1007/s10545-006-0266-3

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN: 0141-8955            Impact factor:   4.982


  6 in total

1.  Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders.

Authors:  F Djouadi; F Aubey; D Schlemmer; J P N Ruiter; R J A Wanders; A W Strauss; Jean Bastin
Journal:  Hum Mol Genet       Date:  2005-08-22       Impact factor: 6.150

2.  Peroxisome proliferator activated receptor delta (PPARdelta) agonist but not PPARalpha corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells.

Authors:  Fatima Djouadi; Flore Aubey; Dimitri Schlemmer; Jean Bastin
Journal:  J Clin Endocrinol Metab       Date:  2004-12-21       Impact factor: 5.958

Review 3.  Fatty acid oxidation disorders.

Authors:  Piero Rinaldo; Dietrich Matern; Michael J Bennett
Journal:  Annu Rev Physiol       Date:  2002       Impact factor: 19.318

Review 4.  The mechanisms of action of PPARs.

Authors:  Joel Berger; David E Moller
Journal:  Annu Rev Med       Date:  2002       Impact factor: 13.739

5.  Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate.

Authors:  Fatima Djouadi; Jean-Paul Bonnefont; Laure Thuillier; Véronique Droin; Noman Khadom; Arnold Munnich; Jean Bastin
Journal:  Pediatr Res       Date:  2003-07-02       Impact factor: 3.756

Review 6.  Genetic defects in fatty acid beta-oxidation and acyl-CoA dehydrogenases. Molecular pathogenesis and genotype-phenotype relationships.

Authors:  Niels Gregersen; Peter Bross; Brage S Andresen
Journal:  Eur J Biochem       Date:  2004-02
  6 in total
  4 in total

1.  Variability in the clinical management of fatty acid oxidation disorders: results of a survey of Canadian metabolic physicians.

Authors:  Beth K Potter; Julian Little; Pranesh Chakraborty; Jonathan B Kronick; Jessica Evans; Julia Frei; Sarah C Sutherland; Kumanan Wilson; Brenda J Wilson
Journal:  J Inherit Metab Dis       Date:  2011-06-01       Impact factor: 4.982

2.  Bezafibrate in skeletal muscle fatty acid oxidation disorders: a randomized clinical trial.

Authors:  Mette Cathrine Ørngreen; Karen Lindhardt Madsen; Nicolai Preisler; Grete Andersen; John Vissing; Pascal Laforêt
Journal:  Neurology       Date:  2014-01-22       Impact factor: 9.910

Review 3.  Treatment Opportunities in Patients With Metabolic Myopathies.

Authors:  Mette Cathrine Ørngreen; John Vissing
Journal:  Curr Treat Options Neurol       Date:  2017-09-21       Impact factor: 3.598

Review 4.  Inborn errors of energy metabolism associated with myopathies.

Authors:  Anibh M Das; Ulrike Steuerwald; Sabine Illsinger
Journal:  J Biomed Biotechnol       Date:  2010-05-26
  4 in total

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