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Journal Abstract Search

245 related items for PubMed ID: 9266218

  • 1. Metabolism of branched-chain amino acids in maple syrup urine disease.
    Schadewaldt P, Wendel U.
    Eur J Pediatr; 1997 Aug; 156 Suppl 1():S62-6. PubMed ID: 9266218
    [Abstract] [Full Text] [Related]

  • 2. 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
    [Abstract] [Full Text] [Related]

  • 3. 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
    [Abstract] [Full Text] [Related]

  • 4. Pathophysiology of maple syrup urine disease: Focus on the neurotoxic role of the accumulated branched-chain amino acids and branched-chain α-keto acids.
    Amaral AU, Wajner M.
    Neurochem Int; 2022 Jul; 157():105360. PubMed ID: 35577033
    [Abstract] [Full Text] [Related]

  • 5. Whole-body L-leucine oxidation in patients with variant form of maple syrup urine disease.
    Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U.
    Pediatr Res; 2001 May; 49(5):627-35. PubMed ID: 11328944
    [Abstract] [Full Text] [Related]

  • 6. Liver transplantation in maple syrup urine disease.
    Wendel U, Saudubray JM, Bodner A, Schadewaldt P.
    Eur J Pediatr; 1999 Dec; 158 Suppl 2():S60-4. PubMed ID: 10603101
    [Abstract] [Full Text] [Related]

  • 7. 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
    [Abstract] [Full Text] [Related]

  • 8. Functional differences in the catabolism of branched-chain L-amino acids in cultured normal and maple syrup urine disease fibroblasts.
    Schadewaldt P, Wendel U.
    Biochem Med Metab Biol; 1989 Apr; 41(2):105-16. PubMed ID: 2719855
    [Abstract] [Full Text] [Related]

  • 9. Quantification of Branched-Chain Amino Acids in Plasma by High-Performance Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS).
    Piri-Moghadam H, Miller A, Pronger D, Vicente F, Charrow J, Haymond S, Lin DC.
    Methods Mol Biol; 2022 Apr; 2546():65-81. PubMed ID: 36127579
    [Abstract] [Full Text] [Related]

  • 10. Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.
    Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B.
    Int J Neurosci; 1994 Nov; 79(1-2):21-45. PubMed ID: 7744549
    [Abstract] [Full Text] [Related]

  • 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
    [Abstract] [Full Text] [Related]

  • 12. Branched-chain α-ketoacid dehydrogenase deficiency (maple syrup urine disease): Treatment, biomarkers, and outcomes.
    Strauss KA, Carson VJ, Soltys K, Young ME, Bowser LE, Puffenberger EG, Brigatti KW, Williams KB, Robinson DL, Hendrickson C, Beiler K, Taylor CM, Haas-Givler B, Chopko S, Hailey J, Muelly ER, Shellmer DA, Radcliff Z, Rodrigues A, Loeven K, Heaps AD, Mazariegos GV, Morton DH.
    Mol Genet Metab; 2020 Mar; 129(3):193-206. PubMed ID: 31980395
    [Abstract] [Full Text] [Related]

  • 13. Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation.
    Bodner-Leidecker A, Wendel U, Saudubray JM, Schadewaldt P.
    J Inherit Metab Dis; 2000 Dec; 23(8):805-18. PubMed ID: 11196106
    [Abstract] [Full Text] [Related]

  • 14. Acute illness in maple syrup urine disease: dynamics of protein metabolism and implications for management.
    Thompson GN, Francis DE, Halliday D.
    J Pediatr; 1991 Jul; 119(1 Pt 1):35-41. PubMed ID: 2066856
    [Abstract] [Full Text] [Related]

  • 15. Assessment of whole body L-leucine oxidation by noninvasive L-[1-13C]leucine breath tests: a reappraisal in patients with maple syrup urine disease, obligate heterozygotes, and healthy subjects.
    Schadewaldt P, Bodner A, Brösicke H, Hammen HW, Wendel U.
    Pediatr Res; 1998 May; 43(5):592-600. PubMed ID: 9585004
    [Abstract] [Full Text] [Related]

  • 16. Treatment of maple syrup urine disease: Benefits, risks, and challenges of liver transplantation.
    Deon M, Guerreiro G, Girardi J, Ribas G, Vargas CR.
    Int J Dev Neurosci; 2023 Oct; 83(6):489-504. PubMed ID: 37340513
    [Abstract] [Full Text] [Related]

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  • 19. Successful pregnancy in maple syrup urine disease: a case report and review of the literature.
    Grünert SC, Rosenbaum-Fabian S, Schumann A, Schwab KO, Mingirulli N, Spiekerkoetter U.
    Nutr J; 2018 May 12; 17(1):51. PubMed ID: 29753318
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