Improved Amino Acid, Bioenergetic Metabolite and Neurotransmitter Profiles following Human Amnion Epithelial Cell Transplant in Intermediate Maple Syrup Urine Disease Mice

► Severe amino acid and neurotransmitter alterations are observed in a mouse model of intermediate Maple Syrup Urine Disease. ► Placental stem cells from human amnion were transplanted into the liver of affected mice. ► Significant and long-term corrections of amino acid and neurotransmitter levels were observed in the periphery as well as the Central Nervous System. ► Human placental stem cells from amnion should be considered for clinical transplant for metabolic liver diseases.