Ácidos Acíclicos/farmacologia; Ácidos Aminoisobutíricos/farmacologia; Barreira Hematoencefálica/metabolismo; Encéfalo/metabolismo; Isoleucina/análogos & derivados; Fenilalanina/metabolismo; Fenilcetonúrias/tratamento farmacológico; Valina/análogos & derivados; Ácidos Acíclicos/administração & dosagem; Ácidos Aminoisobutíricos/administração & dosagem; Animais; Modelos Animais de Doenças; Humanos; Isoleucina/administração & dosagem; Isoleucina/farmacologia; Transportador 1 de Aminoácidos Neutros Grandes/química; Transportador 1 de Aminoácidos Neutros Grandes/metabolismo; Metilação; Camundongos; Camundongos Transgênicos; Terapia de Alvo Molecular; Especificidade de Órgãos; Fenilalanina/sangue; Conformação Proteica; Dobramento de Proteína; Valina/administração & dosagem; Valina/farmacologia

2-(Methylamino)alkanoic acids; 2-aminoisobutyrate (also 2-methyl-2-aminopropionic acid); 3-methyl-2-(methylamino)pentanoic acid; A transporter; AIB; ASC transporter; Ala; Arg; Asp; BBB; BCAA; DOL; GABA; Gln; Glu; Gly; His; Ile; IsoVal; LAT 1-4; LNAA; LNAA transport system, system L transporter; Large neutral amino acid transporter (LAT-1); Large neutral amino acids (LNAA); Leu; MAIB; MePent; MeVal; Met; NPAA; Non-physiological amino acids (NPAA); PBS; PKU; Pah(enu2) mice; Phe; Phenylketonuria (PKU); Thr; Trp; Tyr; Val; X transporter; Y transporter; alanine; arginine; aspartic acid; blood­brain barrier; branched chain amino acids (Leu, Ile, Val); day of life; gamma-aminobutyric acid; glutamic acid; glutamine; glycine; histidine; i.p; intraperitoneal drug administration; isoleucine; isovaline (also 2-methyl-2-aminobutanoic acid); large neutral amino acids; leucine; methionine; methylaminoisobutyrate (also 2-methyl-2-(methylamino)propionic acid); methylvaline (also 3-methyl-2-(methylamino)butanoic acid); murine model of PKU developed with ethylnitrosourea mutagenesis; non-physiological amino acid; phenylalanine; phenylketonuria; phosphate buffered saline; system A (alanine) transport system (small neutral amino acids); system ASC (alanine-serine-cysteine) transport system; system X (acidic amino acid) transporter; system Y (basic amino acid) transporter; threonine; tryptophan; tyrosine; valine