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

Huang, Yan

The PPAR pan-agonist bezafibrate ameliorates cardiomyopathy in a mouse model of Barth syndrome. [electronic resource] - Orphanet journal of rare diseases 03 2017 - 49 p. digital

Publication Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

1750-1172

10.1186/s13023-017-0605-5 doi


Animals
Barth Syndrome--drug therapy
Bezafibrate--therapeutic use
Blotting, Western
Cardiolipins--metabolism
Cardiomyopathies--drug therapy
Disease Models, Animal
Echocardiography
Female
Male
Mice
Peroxisome Proliferator-Activated Receptors--agonists
Polymerase Chain Reaction