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PreKUnil is a medical food formulation consisting of large neutral amino acids (LNAAs) specifically developed for the management of phenylketonuria (PKU). In PKU, elevated blood levels of phenylalanine (Phe) lead to excessive transport of this amino acid into the brain, causing neurotoxicity. PreKUnil works by competitively inhibiting the transport of Phe across the blood-brain barrier via the L-type amino acid transporter 1 (LAT1). By increasing the plasma concentrations of other LNAAs (such as tyrosine, tryptophan, and branched-chain amino acids), the product reduces the brain's uptake of Phe. Furthermore, it serves to supplement precursors for neurotransmitters like dopamine and serotonin (tyrosine and tryptophan), which are often deficient in PKU patients. PreKUnil was an earlier formulation that notably lacked lysine, leading to the subsequent development of modified versions like NeoPhe.
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