Schematic representation of metabolic defects in KPD as revealed by a combination of metabolomics and kinetics. Short black
arrows represent increase or decrease in a metabolite measured in the metabolomics survey, whereas short white arrows represent
increase, decrease, or no change in a metabolite measured in the kinetic studies. Lipid bilayer indicates the boundary of
a liver cell. Dashed long arrows indicate decreased flux, and solid long arrows indicate increased flux. See text for details.
alpha-KG, alpha-ketoglutarate; CPS, carbamoyl phosphate synthetase; GDH, glutamate dehydrogenase.