The Unfolded Protein Response: Integrating Stress Signals Through the Stress Sensor IRE1α

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251

作者:

C HetzF MartinonD RodriguezLH Glimcher

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摘要:

Stress induced by accumulation of unfolded at the (ER) is a classic feature of secretory and is observed in many tissues in diseases including , , , and neurodegeneration. Cellular adaptation to ER stress is achieved by the activation of the unfolded response (UPR), an integrated pathway that transmits information about the status at the ER to the and to restore ER homeostasis. -requiring transmembrane kinase/(α), the most conserved UPR stress sensor, functions as an that processes the mRNA of the transcription -box -1 (). α is a highly regulated process, controlled by the of a dynamic scaffold onto which many regulatory components assemble, here referred to as the UPRosome. Here we provide an overview of the and regulatory mechanisms underlying α function and discuss the emerging role of the UPR in adaptation to stress in specialized secretory and in pathological conditions associated with alterations in ER homeostasis.

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DOI:

10.1152/physrev.00001.2011

被引量:

623

年份:

2011

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