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Methods Enzymol. 2011;491:91-109. doi: 10.1016/B978-0-12-385928-0.00006-7.

Animal models in the study of the unfolded protein response.

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Department of Neurology, Center for Peripheral Neuropathy, The University of Chicago, Chicago, Illinois, USA.


The endoplasmic reticulum, a highly dynamic and complex organelle, is the site for synthesis, folding, and modification of transmembrane and secretory proteins. Any disruptions to the endoplasmic reticulum such as an accumulation of misfolded or unfolded proteins results in activation of the unfolded protein response (UPR). The UPR is comprised of three distinct signal transduction pathways that work to restore homeostasis to the endoplasmic reticulum. This review summarizes select mouse models available to study the UPR and the information learned from the analyses of these models.

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