PPARA and Glycogen storage disease due to glycogenin deficiency: Similarly, in a rodent G6Pase model of the glycogen storage disease, GSD1a, in which patients developed NASH and cirrhosis, the PPARα mixed agonist, bezafibrate, or selective PPARα agonist, fenefibrate, decreased hepatic triglycerides and increased β-oxidation of fatty acids with a concomitant increase in autophagy [71,72].