The PPAR pan-agonist bezafibrate ameliorates cardiomyopathy in a mouse model of Barth syndrome

BACKGROUND: The PGC-1α/PPAR axis has been proposed as a potential therapeutic target for several metabolic disorders. The aim was to evaluate the efficacy of the pan-PPAR agonist, bezafibrate, in tafazzin knockdown mice (TazKD), a mouse model of Barth syndrome that exhibits age-dependent dilated car...

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Veröffentlicht in:Orphanet J Rare Dis
Hauptverfasser: Huang, Yan, Powers, Corey, Moore, Victoria, Schafer, Caitlin, Ren, Mindong, Phoon, Colin K. L., James, Jeanne F., Glukhov, Alexander V., Javadov, Sabzali, Vaz, Frédéric M., Jefferies, John L., Strauss, Arnold W., Khuchua, Zaza
Format: Artigo
Sprache:Inglês
Veröffentlicht: BioMed Central 2017
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Research
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5345250/
https://ncbi.nlm.nih.gov/pubmed/28279226
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13023-017-0605-5
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