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In these diseases, hyperammonemia is accompanied by a severe metabolic acidosis with high anion gap and ketonuria

Science 359:eaao6326 Hu Y, He W, Huang Y, Xiang H, Guo J, Che Y, Cheng X, Hu F, Hu M, Ma T, Yu J, Tian H, Tian S, Ji YX, Zhang P, She ZG, Zhang XJ, Huang Z, Yang J, Li H (2021) Fatty acid synthase-suppressor screening identifies sorting nexin 8 as a therapeutic target for NAFLD