tailieunhanh - Báo cáo khoa học: Increased glucose metabolism and ATP level in brain tissue of Huntington’s disease transgenic mice

Huntington’s disease (HD) is a progressive neurodegenerative disorder characterized by multifarious dysfunctional alterations including mitochon-drial impairment. In the present study, the formation of inclusions caused by the mutation of huntingtin protein and its relationship with changes in energy metabolism and with pathological alterations were investigated both in transgenic and 3-nitropropionic acid-treated mouse models for HD. | ễFEBS Journal Increased glucose metabolism and ATP level in brain tissue of Huntington s disease transgenic mice Judit Olah1 Peter Klivenyi2 Gabriella Gardian2 László Vecsei2 Ferenc Orosz1 Gabor G. Kovacs3 Hans V. Westerhoff4 5 and Judit Ovadi1 1 Institute of Enzymology BiologicalResearch Center Hungarian Academy of Sciences Budapest Hungary 2 Department of Neurology University of Szeged Hungary 3 Institute of Neurology MedicalUniversity Vienna Wien Austria 4 Department of Molecular CellPhysiology Netherlands Institute for Systems Biology Free University Amsterdam Netherlands 5 Manchester Centre for Integrative Systems Biology UK Keywords biosimulation channelling energy metabolism glycolysis activation Huntington s disease Correspondence J. Ovadi Institute of Enzymology Biological Research Center Hungarian Academy of Sciences Karolina ut 29 H-1113 Budapest Hungary Fax 36 1 466 5465 Tel 36 1 279 3129 E-mail ovadi@ Website http ovadi Note The mathematical models described here have been submitted to the Online Cellular Systems Modelling Database and can be accessed free of charge at http . database olah2cc http database olah2cb http . za database olah2hdc http database olah2hdb Received 17 June 2008 revised 17 July 2008 accepted 23 July 2008 doi Huntington s disease HD is a progressive neurodegenerative disorder characterized by multifarious dysfunctional alterations including mitochondrial impairment. In the present study the formation of inclusions caused by the mutation of huntingtin protein and its relationship with changes in energy metabolism and with pathological alterations were investigated both in transgenic and 3-nitropropionic acid-treated mouse models for HD. The HD and normal mice were characterized clinically the affected brain regions were identified by .