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• 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. Funchal C, et al. Neurochem Res. 2004 Apr;29(4):747-53. doi: 10.1023/b:nere.0000018846.66943.30. Neurochem Res. 2004. PMID: 15098937
• 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. Jouvet P, et al. Mol Biol Cell. 2000 May;11(5):1919-32. doi: 10.1091/mbc.11.5.1919. Mol Biol Cell. 2000. PMID: 10793161 Free PMC article.
• 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. Fontella FU, et al. Metab Brain Dis. 2002 Mar;17(1):47-54. doi: 10.1023/a:1014004414733. Metab Brain Dis. 2002. PMID: 11894849
• Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders.

  Xu J, Jakher Y, Ahrens-Nicklas RC. Xu J, et al. Int J Mol Sci. 2020 Oct 11;21(20):7490. doi: 10.3390/ijms21207490. Int J Mol Sci. 2020. PMID: 33050626 Free PMC article. Review.
• Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells.

  Funchal C, Dos Santos AQ, Jacques-Silva MC, Zamoner A, Gottfried C, Wajner M, Pessoa-Pureur R. Funchal C, et al. Metab Brain Dis. 2005 Sep;20(3):205-17. doi: 10.1007/s11011-005-7208-x. Metab Brain Dis. 2005. PMID: 16167198