Protein Misfolding Disorders: A Trip into the ER

Indexed in: Scopus, EBSCO.

Neurodegenerative disorders such as Amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD), Parkinson’s disease (PD), Prion-related disorders (PrD) and Huntington’s disease (HD) share a ...
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The Unfolded Protein Response in Mammalian Cells

Pp. 19-46 (28)

Sung Hoon Back, Justin R. Hassler and Randal J. Kaufman


The endoplasmic reticulum (ER) is a well-orchestrated factory where secretory and membrane proteins are manufactured, modified and correctly folded prior to trafficking to their final destinations. Conditions that interfere with ER function lead to an evolutionarily conserved cellular stress response termed the unfolded protein response (UPR). The UPR initially transduces signals to reestablish ER homeostasis but with prolonged or acute ER stress these signals undergo a transition that initiate cell death. In this review, we summarize our current understanding of the mammalian UPR and discuss the cell-type and tissue-specific functions of UPR components revealed by studies from genetic mouse models.


Howard Hughes Medical Institute, University of Michigan Medical Center, Ann Arbor, MI 48109-0650 USA.