Effects of supplementation on food intake, body weight and hepatic metabolites in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse model of human citrin deficiency

► Mature Ctrn/mGPD-KO mice lose weight when switched to a purified maintenance diet. ► The loss of body weight from reduced food intake reverted with increased protein. ► Addition of amino acids, pyruvate or MCT also increased food intake and body weight. ► Sucrose gavage leads to hyperammonemia and altered hepatic metabolites in the mice. ► Addition of supplements to sucrose gavage resulted in distinct metabolic correction.