Neurochem Int 2021 May 6;145:104984. Epub 2021 Feb 6.
Department of Biomedical and Molecular Sciences, Queen's University, Kingston, Ontario, K7L 3N6, Canada; Department of Anesthesiology & Perioperative Medicine, Queen's University, Kingston, Ontario, K7L 3N6, Canada; Centre for Neuroscience Studies, Queen's University, Kingston, Ontario, K7L 3N6, Canada. Electronic address:
Arginase-1 (Arg1) is an enzyme controlling the final step of the urea cycle, with highest expression in the liver and lower expression in the lungs, pancreas, kidney, and some blood cells. Arg1 deficiency is an inherited urea cycle disorder presenting with neurological dysfunction including spastic diplegia, intellectual and growth retardation, and encephalopathy. The contribution of Arg1 expression in the central and peripheral nervous system to the development of neurological phenotypes remains largely unknown. Read More