Huntigton’s Disease (HD) is a genetic disorder characterized by the progressive degeneration of neurons resulting in involuntary movement and progressive cell death. Patients with HD express a mutant Huntingtin protein containing an extended polyglutamine (polyQ) trait that favors the formation of Htt-aggregates. Patients with polyQ diseases like Huntington’s Disease and Spinocerebellar Ataxias (SCAs)were found partially protected against the development of cancer. Lancet Oncol. 2012 Jun;13(6):642-8 suggesting that might have reduced growth factor signaling. In addition, Glutamine synthetase (GS1) activity was found reduced in choreic brain post mortem. Life Sci.1983 Apr 25;32:1949-55. Life Sci.1982 Sep 13;31:1151-9. So we asked if there was a possible link between the function of the Glutamate Cycle and Growth Factor signaling in neurons and glia cells expressing the mutant human HTTQ93 polyQ protein?
Glutamine synthetase induces autophagy and neuronal survival in a Drosophila model of Huntington’s Disea / L. Vernizzi, C. Paiardi, M.E. Pasini, A.M. Valenza, G. Licata, T. Vitali, M.A. Vanoni, C. Gellera, M. Rizzetto, F. Taroni, C. Mariotti, P. Bellosta. ((Intervento presentato al convegno EHDN tenutosi a Aia nel 2016.
Glutamine synthetase induces autophagy and neuronal survival in a Drosophila model of Huntington’s Disea
C. PaiardiSecondo
;M.E. Pasini;A.M. Valenza;M.A. Vanoni;
2016
Abstract
Huntigton’s Disease (HD) is a genetic disorder characterized by the progressive degeneration of neurons resulting in involuntary movement and progressive cell death. Patients with HD express a mutant Huntingtin protein containing an extended polyglutamine (polyQ) trait that favors the formation of Htt-aggregates. Patients with polyQ diseases like Huntington’s Disease and Spinocerebellar Ataxias (SCAs)were found partially protected against the development of cancer. Lancet Oncol. 2012 Jun;13(6):642-8 suggesting that might have reduced growth factor signaling. In addition, Glutamine synthetase (GS1) activity was found reduced in choreic brain post mortem. Life Sci.1983 Apr 25;32:1949-55. Life Sci.1982 Sep 13;31:1151-9. So we asked if there was a possible link between the function of the Glutamate Cycle and Growth Factor signaling in neurons and glia cells expressing the mutant human HTTQ93 polyQ protein?
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