The unfolded protein response: mechanisms and therapy of neurodegeneration
نویسندگان
چکیده
منابع مشابه
The unfolded protein response: mechanisms and therapy of neurodegeneration
Activation of the unfolded protein response is emerging as a common theme in protein-misfolding neurodegenerative diseases, with relevant markers observed in patient tissue and mouse models. Genetic and pharmacological manipulation of the pathway in several mouse models has shown that this is not a passive consequence of the neurodegeneration process. Rather, overactivation of the protein kinas...
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Proteostasis in the lumen of the endoplasmic reticulum is defended by signalling pathways that match the load of unfolded proteins to the complement of chaperones in the organelle. This balancing act is attained via a transcriptional program that activates genes encoding chaperones and other proteins that function in the secretory pathway and a translational program that transiently attenuates ...
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Where does the UPR function? Between the endoplasmic reticulum (ER) and the nucleus of eukaryotic cells. All secreted proteins and proteins that reside in secretory compartments translocate as nascent peptide chains into the ER, where they may undergo folding, modification and assembly before assuming their functional conformations. The ER maintains a specialized oxidizing environment to aid ch...
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ژورنال
عنوان ژورنال: Brain
سال: 2016
ISSN: 0006-8950,1460-2156
DOI: 10.1093/brain/aww101