Effect of Leucine Administration on Creatine Kinase Activity in Rat Brain

作者: Carmen Pilla , Rui Felipe de Oliveira Cardozo , Carlos Severo Dutra-Filho , Angela Terezinha Souza Wyse , Moacir Wajner

DOI: 10.1023/A:1021974517837

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摘要: Maple syrup urine disease (MSUD) is a metabolic disorder biochemically characterized by the accumulation of branched-chain amino acids (BCAA) and their keto (BCKA) in blood tissues. Neurological dysfunction usually present patients, but pathophysiology brain damage still obscure. Considering that energy metabolism possibly altered MSUD, main objective this study was to determine creatine kinase activity rats subjected acute chronic administration leucine. Chronic hyperleucinemia induced subcutaneous administrations 4.8 μmol leucine/g body weight, twice day, from 6th 21st postnatal day. For hyperleucinemia, 21-day-old received three acid at 3 h interval. Twelve hours after treatment or 1 one, were killed measured. The results indicated leucine leucine-treated rats. crucial role plays homeostasis brain, if these effects also occur MSUD it possible alteration enzyme may contribute found disease.

参考文章(32)
Fernanda U. Fontella, Edson Gassen, Vânia Pulrolnik, Clóvis M.D. Wannmacher, Adriane B. Klein, Moacir Wajner, Carlos S. Dutra-Filho, Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease. Metabolic Brain Disease. ,vol. 17, pp. 47- 54 ,(2002) , 10.1023/A:1014004414733
PA Harper, PJ Healy, JA Dennis, None, Maple syrup urine disease (branched chain ketoaciduria). American Journal of Pathology. ,vol. 136, pp. 1445- 1447 ,(1990)
John H. Menkes, MAPLE SYRUP DISEASE Isolation and Identification of Organic Acids in the Urine Pediatrics. ,vol. 23, pp. 348- 353 ,(1959)
H. Ronald Zielke, Yinyin Huang, Peter J. Baab, Roger M. Collins, Jr., Carol L. Zielke, J. Tyson Tildon, Effect of α-Ketoisocaproate and Leucine on the in Vivo Oxidation of Glutamate and Glutamine in the Rat Brain Neurochemical Research. ,vol. 22, pp. 1159- 1164 ,(1997) , 10.1023/A:1027325620983
A. Lewis Farr, Oliver H. Lowry, Rose J. Randall, Nira J. Rosebrough, Protein Measurement with the Folin Phenol Reagent Journal of Biological Chemistry. ,vol. 193, pp. 265- 275 ,(1951)
James J. Riviello, Iraj Rezvani, Angelo M. DiGeorge, Catherine M. Foley, Cerebral edema causing death in children with maple syrup urine disease. The Journal of Pediatrics. ,vol. 119, pp. 42- 45 ,(1991) , 10.1016/S0022-3476(05)81036-4
Hidekazu Tomimoto, Kazumi Yamamoto, HenryA. Homburger, Takehiko Yanagihara, Immunoelectron microscopic investigation of creatine kinase BB-isoenzyme after cerebral ischemia in gerbils. Acta Neuropathologica. ,vol. 86, pp. 447- 455 ,(1993) , 10.1007/BF00228579
Y. Huang, H. Ronald Zielke, J. Tyson Tildon, Carol L. Zielke, Peter J. Baab, Elevation of Amino Acids in the Interstitial Space of the Rat Brain following Infusion of Large Neutral Amino and Keto Acids by Microdialysis: Leucine Infusion Developmental Neuroscience. ,vol. 18, pp. 415- 419 ,(1996) , 10.1159/000111435
Michael Aksenov, Marina Aksenova, D. Allan Butterfield, William R. Markesbery, Oxidative modification of creatine kinase BB in Alzheimer's disease brain. Journal of Neurochemistry. ,vol. 74, pp. 2520- 2527 ,(2002) , 10.1046/J.1471-4159.2000.0742520.X