FIGURE 6.

Model of LRP130 action and effect on energy metabolism. LRP130 interacts with POLRMT, a component of the initiation complex, to activate mitochondrial transcription. Polypeptides encoded by mtDNA are increased, promoting supercomplexes and OXPHOS. Metabolic pathways dependent on cycling of coenzymes by the electron transport chain are stimulated by increased OXPHOS. In summary, increases in mitochondrial transcription by LRP130 are associated with remodeling of mitochondria and enhanced oxidative metabolism, notably hepatic FAO and ketogenesis.