Targeting unfolded protein response signaling pathways to ameliorate protein misfolding diseases.

作者: Lisa M Ryno , R Luke Wiseman , Jeffery W Kelly

DOI: 10.1016/J.CBPA.2013.04.009

关键词: Protein foldingTranslational attenuationProteostasisHomeostasisBiologySignal transductionCell biologyUnfolded protein responseEndoplasmic reticulumProtein biosynthesis

摘要: Protein homeostasis (or proteostasis) within the endoplasmic reticulum (ER) is regulated by unfolded protein response (UPR). The UPR consists of three integrated signaling pathways activated accumulation misfolded proteins ER lumen. Activation alters proteostasis through translational attenuation new synthesis and transcriptional remodeling pathways, providing a mechanism to adapt in cellular stress. capacity alter suggests that exogenous manipulation offers therapeutic promise fate pathologic associated with human misfolding diseases. Here, we discuss potential activation treat disease highlight specific small molecule approaches for regulating could be beneficial

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