242 related articles for article (PubMed ID: 6422161)
1. Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease.
Shigematsu Y; Kikuchi K; Momoi T; Sudo M; Kikawa Y; Nosaka K; Kuriyama M; Haruki S; Sanada K; Hamano N
J Inherit Metab Dis; 1983; 6(4):183-9. PubMed ID: 6422161
[TBL] [Abstract][Full Text] [Related]
2. Exchange transfusion in acute episodes of maple syrup urine disease. Studies on branched-chain amino and keto acids.
Wendel U; Langenbeck U; Lombeck I; Bremer HJ
Eur J Pediatr; 1982 Jul; 138(4):293-6. PubMed ID: 7128634
[TBL] [Abstract][Full Text] [Related]
3. Maple syrup urine disease: treatment of the acutely ill newborn.
Hammersen G; Wille L; Schmidt H; Lutz P; Bickel H
Eur J Pediatr; 1978 Oct; 129(3):157-65. PubMed ID: 699921
[TBL] [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
[TBL] [Abstract][Full Text] [Related]
5. Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.
Wajner A; Bürger C; Dutra-Filho CS; Wajner M; de Souza Wyse AT; Wannmacher CM
Metab Brain Dis; 2007 Mar; 22(1):77-88. PubMed ID: 17295076
[TBL] [Abstract][Full Text] [Related]
6. Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.
Langenbeck U; Wendel U; Mench-Hoinowski A; Kuschel D; Becker K; Przyrembel H; Bremer HJ
Clin Chim Acta; 1978 Sep; 88(2):283-91. PubMed ID: 699323
[TBL] [Abstract][Full Text] [Related]
7. The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease.
Snyderman SE; Goldstein F; Sansaricq C; Norton PM
Pediatr Res; 1984 Sep; 18(9):851-3. PubMed ID: 6483508
[TBL] [Abstract][Full Text] [Related]
8. Blood and tissue branched-chain amino and alpha-keto acid concentrations: effect of diet, starvation, and disease.
Hutson SM; Harper AE
Am J Clin Nutr; 1981 Feb; 34(2):173-83. PubMed ID: 7211722
[TBL] [Abstract][Full Text] [Related]
9. Diagnosis and treatment of maple syrup disease: a study of 36 patients.
Morton DH; Strauss KA; Robinson DL; Puffenberger EG; Kelley RI
Pediatrics; 2002 Jun; 109(6):999-1008. PubMed ID: 12042535
[TBL] [Abstract][Full Text] [Related]
10. Total parenteral nutrition therapy of toxic maple syrup urine disease.
Townsend I; Kerr DS
Am J Clin Nutr; 1982 Aug; 36(2):359-65. PubMed ID: 6808824
[TBL] [Abstract][Full Text] [Related]
11. Peritoneal dialysis in maple-syrup-urine disease: studies on branched-chain amino and keto acids.
Wendel U; Becker K; Przyrembel H; Bulla M; Manegold C; Mench-Hoinowski A; Langenbeck U
Eur J Pediatr; 1980 Jun; 134(1):57-63. PubMed ID: 7408911
[No Abstract] [Full Text] [Related]
12. Thiamine response in maple syrup urine disease.
Fernhoff PM; Lubitz D; Danner DJ; Dembure PP; Schwartz HP; Hillman R; Bier DM; Elsas LJ
Pediatr Res; 1985 Oct; 19(10):1011-6. PubMed ID: 3903643
[TBL] [Abstract][Full Text] [Related]
13. Clearance of branched chain amino acids by peritoneal dialysis in maple syrup urine disease.
McMahon Y; MacDonnell RC
Adv Perit Dial; 1990; 6():31-4. PubMed ID: 1982835
[TBL] [Abstract][Full Text] [Related]
14. Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease.
Reis M; Farage M; Wolosker H
Biochim Biophys Acta; 2000 Jul; 1475(2):114-8. PubMed ID: 10832024
[TBL] [Abstract][Full Text] [Related]
15. Prevention of DNA damage by L-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro.
Mescka CP; Wayhs CA; Guerreiro G; Manfredini V; Dutra-Filho CS; Vargas CR
Gene; 2014 Sep; 548(2):294-8. PubMed ID: 25046137
[TBL] [Abstract][Full Text] [Related]
16. Plasma amino acid analyses in two cases of maple syrup urine disease.
Surarit R; Srisomsap C; Wasant P; Svasti J; Suthatvoravut U; Chokchaichamnankit D; Liammongkolkul S
Southeast Asian J Trop Med Public Health; 1999; 30 Suppl 2():138-9. PubMed ID: 11400750
[TBL] [Abstract][Full Text] [Related]
17. Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease.
Fontella FU; Gassen E; Pulrolnik V; Wannmacher CM; Klein AB; Wajner M; Dutra-Filho CS
Metab Brain Dis; 2002 Mar; 17(1):47-54. PubMed ID: 11894849
[TBL] [Abstract][Full Text] [Related]
18. Branched chain amino acids induce apoptosis in neural cells without mitochondrial membrane depolarization or cytochrome c release: implications for neurological impairment associated with maple syrup urine disease.
Jouvet P; Rustin P; Taylor DL; Pocock JM; Felderhoff-Mueser U; Mazarakis ND; Sarraf C; Joashi U; Kozma M; Greenwood K; Edwards AD; Mehmet H
Mol Biol Cell; 2000 May; 11(5):1919-32. PubMed ID: 10793161
[TBL] [Abstract][Full Text] [Related]
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]
20. Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.
Funchal C; Latini A; Jacques-Silva MC; Dos Santos AQ; Buzin L; Gottfried C; Wajner M; Pessoa-Pureur R
Neurochem Int; 2006 Dec; 49(7):640-50. PubMed ID: 16822590
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]