Reversing Neurodegeneration:A Promise Unfolds Harry T Orr, Huda Y Zoghbi Cell Volume 101, Issue 1, Pages 1-4 (March 2000) DOI: 10.1016/S0092-8674(00)80617-0 Copyright © 2000 Cell Press Terms and Conditions
Figure 1 A Model of Polyglutamine-Induced Neurodegeneration and Its Reversal (A) Expression of a mutant peptide with expanded polyglutamine (Q) tract leads to neuronal degeneration and eventual death. Note that both aggregated and solitary protein induce neuronal dysfunction that precedes death. (B) Turning off mutant protein expression gives the neuron enough of a reprieve to clear the mutant protein by the chaperone/ubiquitin-proteasome pathway (UPP) and subsequently recover at least some function. Cell 2000 101, 1-4DOI: (10.1016/S0092-8674(00)80617-0) Copyright © 2000 Cell Press Terms and Conditions