A liver-specific defect of Acyl-CoA degradation produces hyperammonemia, hypoglycemia and a distinct hepatic Acyl-CoA pattern.
Gauthier N, Wu JW, Wang SP, Allard P, Mamer OA, Sweetman L, Moser AB, Kratz L, Alvarez F, Robitaille Y, et al. PLoS One. 2013; 8(7):e60581. Epub 2013 Jul 5.