Complementary dietary treatment using lysine-free, arginine-fortified amino acid supplements in glutaric aciduria type I - A decade of experience

► Arginine supplementation reduces the cerebral lysine influx and oxidation. ► This treatment limits the accumulation of neurotoxins in glutaric aciduria type I. ► Arginine-fortified diet improves the outcome in glutaric aciduria type I. ► Arginine fortification adds to treatment according to guideline recommendations. ► Exploitation of the blood brain barrier is a promising therapeutic strategy.