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Journal Abstract Search

120 related items for PubMed ID: 6034241

  • 1. Further observations on the biochemical lesion in maple syrup urine disease.
    Dreyfus PM, Prensky AL.
    Nature; 1967 Apr 15; 214(5085):276. PubMed ID: 6034241
    [No Abstract] [Full Text] [Related]

  • 2. Transaminations between amino acids and keto acids elevated in phenylketonuria and maple syrup urine disease.
    Lees GJ, Weiner N.
    J Neurochem; 1973 Feb 15; 20(2):389-403. PubMed ID: 4698286
    [No Abstract] [Full Text] [Related]

  • 3. Branched chain alpha-keto acid metabolism. I. Isolation, purification, and partial characterization of bovine liver alpha-ketoisocaproic:alpha-keto-beta-methylvaleric acid dehydrogenase.
    Connelly JL, Danner DJ, Bowden JA.
    J Biol Chem; 1968 Mar 25; 243(6):1198-203. PubMed ID: 5689906
    [No Abstract] [Full Text] [Related]

  • 4. Enzyme activity in classical and variant forms of maple syrup urine disease.
    Dancis J, Hutzler J, Snyderman SE, Cox RP.
    J Pediatr; 1972 Aug 25; 81(2):312-20. PubMed ID: 5042489
    [No Abstract] [Full Text] [Related]

  • 5. The enzyme defect in maple syrup urine disease (branched chain ketoaciduria).
    Seegmiller JE, Westall RG.
    J Ment Defic Res; 1967 Dec 25; 11(4):288-94. PubMed ID: 5582930
    [No Abstract] [Full Text] [Related]

  • 6. 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 25; 22(1):77-88. PubMed ID: 17295076
    [Abstract] [Full Text] [Related]

  • 7. [A case of maple syrup urine disease. Biochemical study].
    Lamedica GM, Fregonese B, Moroni G, Famularo L.
    Minerva Pediatr; 1972 Dec 15; 24(43):2143-9. PubMed ID: 4652219
    [No Abstract] [Full Text] [Related]

  • 8. Reduction of glutamate uptake into cerebral cortex of developing rats by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.
    Funchal C, Rosa AM, Wajner M, Wofchuk S, Pureur RP.
    Neurochem Res; 2004 Apr 15; 29(4):747-53. PubMed ID: 15098937
    [Abstract] [Full Text] [Related]

  • 9. Classical maple syrup urine disease: cofactor resistance.
    Elsas LJ, Pask BA, Wheeler FB, Perl DP, Truster S.
    Metabolism; 1972 Oct 15; 21(10):929-44. PubMed ID: 4342010
    [No Abstract] [Full Text] [Related]

  • 10. The inhibition of pyruvate decarboxylation in rat brain by -ketoisocaproic acid.
    Bowden JA, McArthur CL, Fried M.
    Biochem Med; 1971 Apr 15; 5(2):101-8. PubMed ID: 5167175
    [No Abstract] [Full Text] [Related]

  • 11. Biochemical studies on a variant of branched chain ketoaciduria in a 19-year-old female.
    Fischer MH, Gerritsen T.
    Pediatrics; 1971 Nov 15; 48(5):795-801. PubMed ID: 5125877
    [No Abstract] [Full Text] [Related]

  • 12. Metabolism of 5-hydroxy-4-keto-valeric acid in the rat.
    Wang FK, Koch J, Stokstad EL.
    Biochem Biophys Res Commun; 1970 Aug 11; 40(3):576-82. PubMed ID: 5492154
    [No Abstract] [Full Text] [Related]

  • 13. Intermittent branched-chain ketonuria. Variant of maple-syrup-urine disease.
    Dancis J, Hutzler J, Rokkones T.
    N Engl J Med; 1967 Jan 12; 276(2):84-9. PubMed ID: 6015521
    [No Abstract] [Full Text] [Related]

  • 14. STUDIES ON CARBON DIOXIDE FIXATION IN NORMAL AND ALLOXAN-DIABETIC ANIMALS.
    WAGLE SR, ASHMORE J.
    Biochim Biophys Acta; 1963 Aug 13; 74():564-5. PubMed ID: 14071606
    [No Abstract] [Full Text] [Related]

  • 15. Inhibition, by 2-oxo acids that accumulate in maple-syrup-urine disease, of lactate, pyruvate, and 3-hydroxybutyrate transport across the blood-brain barrier.
    Cremer JE, Teal HM, Cunningham VJ.
    J Neurochem; 1982 Sep 13; 39(3):674-7. PubMed ID: 7201506
    [Abstract] [Full Text] [Related]

  • 16. Heterogeneity in maple syrup urine disease: aspects of cofactor requirement and complementation in cultured fibroblasts.
    Singh S, Willers I, Goedde HW.
    Clin Genet; 1977 Apr 13; 11(4):277-84. PubMed ID: 192504
    [Abstract] [Full Text] [Related]

  • 17. Complementation analysis of maple syrup urine disease in heterokaryons derived from cultured human fibroblasts.
    Lyons LB, Cox RP, Dancis J.
    Nature; 1973 Jun 29; 243(5409):533-5. PubMed ID: 4355237
    [No Abstract] [Full Text] [Related]

  • 18. Evidence that the branched-chain alpha-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex.
    Funchal C, Gottfried C, De Almeida LM, Wajner M, Pessoa-Pureur R.
    Glia; 2004 Nov 15; 48(3):230-40. PubMed ID: 15390119
    [Abstract] [Full Text] [Related]

  • 19. [Biochemical studies in a patient with maple syrup urine disease (author's transl)].
    Vaca G, Rivas F, Sánchez-Corona J, Olivares N, Aguirre-Negrete MG, González-Quiroga G, Medina C, Hernández A, Cantú JM.
    Rev Invest Clin; 1981 Nov 15; 33(4):379-82. PubMed ID: 7330511
    [No Abstract] [Full Text] [Related]

  • 20. DETECTION OF THE HETEROZYGOTE IN MAPLE SYRUP URINE DISEASE.
    DANCIS J, HUTZLER J, LEVITZ M.
    J Pediatr; 1965 Mar 15; 66():595-603. PubMed ID: 14264316
    [No Abstract] [Full Text] [Related]

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