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HD INSIGHTS HD Research in Chile, cont... General pharmacological strategies may include administration of chemical chaperones such as TUDCA or 4-PBA, modulators of UPR components (i.e. available IRE1 inhibitors), or autophagy enhancers such as trehalose or rapamycin. The use of gene therapy to target protein- folding stress has been applied to other neurodegenerative diseases 1 Vidal R, Caballero B, Couve A, Hetz C. Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's disease. Curr Mol Med. 2011 Feb;11(1):1-12. with the aim of targeting protein homeostasis, and may be an approach to consider, since HD is a disease that progresses slowly. The development of network- modifying therapeutic interventions may lead to important protective advances in the HD field. Minor shifts in the protein homeostasis network 2 Lee H, Noh JY, Oh Y, et al. IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux. Hum Mol Genet 2012 Jan;21(1): 101-14. may involve the alteration of hundreds of target genes that as a whole may result in beneficial effects in HD. Thus, modulating global homeostatic processes could have broad impact for chronic alterations observed in HD that involve multiple aspects of neuronal physiology and protein homeostasis. 3 Vidal RL, Figueroa A, Court FA, et al. Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy. Hum Mol Genet 2012 May;21(10):2245-62. If you are interested in contributing a piece that highlights research in your part of the world, please contact us at editor@hdinsights.org. Editorial Board Members Ray Dorsey, MD — Editor, Johns Hopkins University Donald Kennedy, PhD — Stanford University Nobuyuki Nukina, MD, PhD — Riken Brain Science Institute Rodrigo Osorio — Agrupacion Chilena de Huntington Bernard Ravina, MD — Biogen Idec Ralf Reilmann, MD — University of Munster Sarah Tabrizi, MBChB, PhD — University College London Leslie Thompson, PhD — University of California Irvine Huntington Study Group Shari Kinel, JD — HSG Executive Director Liz McCarthy, BA — HSG Administrative Manager Jillian Lowell, BS — HSG Event Planner Contributors Claudio Hetz, PhD Emily Mitchell Sontag, PhD Lise Munsie, PhD Paige Nichols, BA Rene Vidal, PhD Publication Staff Kristin Darwin, BS — Deputy Editor Robin Taylor — Production Editor Martin Holmes — Technical Editor Todd Bernhard — Copy Editor Vinayak Venkataraman, BS — Website Designer Dave Kolko — Distribution Specialist For more information on HD Insights, or to receive a free subscription, please visit us at www.HDInsights.org or email us at editor@hdinsights.org. 12 Copyright © Huntington Study Group 2012. All rights reserved. HD Insights, Vol. 3