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5. ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease. Wu JY; Kao HJ; Li SC; Stevens R; Hillman S; Millington D; Chen YT J Clin Invest; 2004 Feb; 113(3):434-40. PubMed ID: 14755340 [TBL] [Abstract][Full Text] [Related]
6. Total branched-chain amino acids requirement in patients with maple syrup urine disease by use of indicator amino acid oxidation with L-[1-13C]phenylalanine. Riazi R; Rafii M; Clarke JT; Wykes LJ; Ball RO; Pencharz PB Am J Physiol Endocrinol Metab; 2004 Jul; 287(1):E142-9. PubMed ID: 14970005 [TBL] [Abstract][Full Text] [Related]
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11. Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease. Vilela TC; Scaini G; Furlanetto CB; Pasquali MA; Santos JP; Gelain DP; Moreira JC; Schuck PF; Ferreira GC; Streck EL Metab Brain Dis; 2017 Feb; 32(1):115-122. PubMed ID: 27510712 [TBL] [Abstract][Full Text] [Related]
12. Deficiency of BCAT2-mediated branched-chain amino acid catabolism promotes colorectal cancer development. Kang ZR; Jiang S; Han JX; Gao Y; Xie Y; Chen J; Liu Q; Yu J; Zhao X; Hong J; Chen H; Chen YX; Chen H; Fang JY Biochim Biophys Acta Mol Basis Dis; 2024 Feb; 1870(2):166941. PubMed ID: 37926361 [TBL] [Abstract][Full Text] [Related]
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15. Administration of branched-chain amino acids alters epigenetic regulatory enzymes in an animal model of Maple Syrup Urine Disease. Streck EL; Bussular FP; Wessler LB; Duarte MB; Rezende VL; Rodrigues MS; Torres CA; Lemos IS; Candiotto G; Gava FF; de Oliveira J; Valvassori SS Metab Brain Dis; 2021 Feb; 36(2):247-254. PubMed ID: 33098071 [TBL] [Abstract][Full Text] [Related]
16. A molecular model of human branched-chain amino acid metabolism. Suryawan A; Hawes JW; Harris RA; Shimomura Y; Jenkins AE; Hutson SM Am J Clin Nutr; 1998 Jul; 68(1):72-81. PubMed ID: 9665099 [TBL] [Abstract][Full Text] [Related]
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19. Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. Schadewaldt P; Hammen HW; Ott AC; Wendel U J Inherit Metab Dis; 1999 Aug; 22(6):706-22. PubMed ID: 10472531 [TBL] [Abstract][Full Text] [Related]
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