Abstract 2619 Lessons from a rare disease: Glycerol-3-phosphate activates ChREBP, FGF21 transcription, and lipogenesis in a mouse model of Citrin Deficiency

The malate-aspartate shuttle (MAS) is the major system to move high energy electrons from hepatocytosolic NADH to the mitochondrial matrix. The MAS consists of two antiporters on the mitochondrial membrane plus isozymes of aspartate aminotransferase and malate dehydrogenase on both sides of the membrane. Electrons from NADH are carried across the membrane on malate in exchange for alpha-ketoglutarate. The antiporter that moves Glu into the mitochondrial matrix against Asp into cytosol is encoded by the SLC25A13 gene, which is inactivated in Citrin Deficiency (CD), one of the most common monogenic diseases in the world.