BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

140 related articles for article (PubMed ID: 37494004)

  • 1. Oral enzyme therapy for maple syrup urine disease (MSUD) suppresses plasma leucine levels in intermediate MSUD mice and healthy nonhuman primates.
    Skvorak K; Liu J; Kruse N; Mehmood R; Das S; Jenne S; Chng C; Lao UL; Duan D; Asfaha J; Du F; Teadt L; Sero A; Ching C; Riggins J; Pope L; Yan P; Mashiana H; Ismaili MHA; McCluskie K; Huisman G; Silverman AP
    J Inherit Metab Dis; 2023 Nov; 46(6):1089-1103. PubMed ID: 37494004
    [TBL] [Abstract][Full Text] [Related]  

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

  • 3. Maple syrup urine disease decompensation misdiagnosed as a psychotic event.
    Higashimoto T; Whitehead MT; MacLeod E; Starin D; Regier DS
    Mol Genet Metab Rep; 2022 Sep; 32():100886. PubMed ID: 35756860
    [TBL] [Abstract][Full Text] [Related]  

  • 4. 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
    [TBL] [Abstract][Full Text] [Related]  

  • 5. 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
    [TBL] [Abstract][Full Text] [Related]  

  • 6. 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
    [TBL] [Abstract][Full Text] [Related]  

  • 7. Intravenous administration of a branched-chain amino-acid-free solution in children and adults with acute decompensation of maple syrup urine disease: a prospective multicentre observational study.
    Alili JM; Berleur MP; Husson MC; Mention K; Schiff M; Arnoux JB; Brassier A; Guemman AS; Grisel C; Dubois S; Abi-Wardé MT; Broissand C; Servais A; Dao M; de Lonlay P
    Orphanet J Rare Dis; 2022 May; 17(1):202. PubMed ID: 35578286
    [TBL] [Abstract][Full Text] [Related]  

  • 8. 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
    [TBL] [Abstract][Full Text] [Related]  

  • 9. Production and characterization of murine models of classic and intermediate maple syrup urine disease.
    Homanics GE; Skvorak K; Ferguson C; Watkins S; Paul HS
    BMC Med Genet; 2006 Mar; 7():33. PubMed ID: 16579849
    [TBL] [Abstract][Full Text] [Related]  

  • 10. Muscle-directed AAV gene therapy rescues the maple syrup urine disease phenotype in a mouse model.
    Greig JA; Jennis M; Dandekar A; Chorazeczewski JK; Smith MK; Ashley SN; Yan H; Wilson JM
    Mol Genet Metab; 2021; 134(1-2):139-146. PubMed ID: 34454844
    [TBL] [Abstract][Full Text] [Related]  

  • 11. 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; 17(1):51. PubMed ID: 29753318
    [TBL] [Abstract][Full Text] [Related]  

  • 12. Markers associated with inborn errors of metabolism of branched-chain amino acids and their relevance to upper levels of intake in healthy people: an implication from clinical and molecular investigations on maple syrup urine disease.
    Mitsubuchi H; Owada M; Endo F
    J Nutr; 2005 Jun; 135(6 Suppl):1565S-70S. PubMed ID: 15930470
    [TBL] [Abstract][Full Text] [Related]  

  • 13. Clues and challenges in the diagnosis of intermittent maple syrup urine disease.
    Pode-Shakked N; Korman SH; Pode-Shakked B; Landau Y; Kneller K; Abraham S; Shaag A; Ulanovsky I; Daas S; Saraf-Levy T; Reznik-Wolf H; Vivante A; Pras E; Almashanu S; Anikster Y
    Eur J Med Genet; 2020 Jun; 63(6):103901. PubMed ID: 32151765
    [TBL] [Abstract][Full Text] [Related]  

  • 14. Treatment of maple syrup urine disease with high flow hemodialysis in a neonate.
    Aygün F; Kıykım E; Aktuğlu-Zeybek Ç; Zubarioğlu T; Cam H
    Turk J Pediatr; 2019; 61(1):107-110. PubMed ID: 31559730
    [TBL] [Abstract][Full Text] [Related]  

  • 15. Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease.
    Zinnanti WJ; Lazovic J; Griffin K; Skvorak KJ; Paul HS; Homanics GE; Bewley MC; Cheng KC; Lanoue KF; Flanagan JM
    Brain; 2009 Apr; 132(Pt 4):903-18. PubMed ID: 19293241
    [TBL] [Abstract][Full Text] [Related]  

  • 16. 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
    [TBL] [Abstract][Full Text] [Related]  

  • 17. Oxidative stress in plasma from maple syrup urine disease patients during treatment.
    Barschak AG; Sitta A; Deon M; Barden AT; Dutra-Filho CS; Wajner M; Vargas CR
    Metab Brain Dis; 2008 Mar; 23(1):71-80. PubMed ID: 18026828
    [TBL] [Abstract][Full Text] [Related]  

  • 18. 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; 2546():65-81. PubMed ID: 36127579
    [TBL] [Abstract][Full Text] [Related]  

  • 19. Melatonin improves behavioral parameters and oxidative stress in zebrafish submitted to a leucine-induced MSUD protocol.
    Duarte MB; Medeiros BZ; da Silva Lemos I; da Silva GL; Alano CG; Dondossola ER; Torres CA; Effting PS; Rico EP; Streck EL
    Metab Brain Dis; 2023 Aug; 38(6):2105-2114. PubMed ID: 37099078
    [TBL] [Abstract][Full Text] [Related]  

  • 20.
    ; ; . PubMed ID:
    [No Abstract]   [Full Text] [Related]  

    [Next]    [New Search]
    of 7.