کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
6262285 1292347 2016 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
The stress of prion disease
ترجمه فارسی عنوان
استرس بیماری پریون
کلمات کلیدی
پریون، استرس تناسلی اندوپلاسمی، شاپورونهای مولکولی، پاسخ پروتئین باز شده،
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی علوم اعصاب (عمومی)
چکیده انگلیسی


- Endoplasmic reticulum stress is a prominent feature in prion diseases.
- Several complex and inter-related pathways respond to ER stress induced by prion accumulation.
- Chronic and unresolved ER stress leads to neuronal dysfunction and death.
- Further knowledge on the ER stress pathways may open new avenues for therapeutic intervention.

Prion diseases are fatal neurodegenerative disorders that include scrapie of sheep, bovine spongiform encephalopathy of cattle, chronic wasting disease of cervids, and Creutzfeldt-Jakob disease (CJD) of humans. The etiology for prion diseases can be infectious, sporadic, or hereditary. However, the common denominator for all types is the formation of a transmissible agent composed of a β-sheet-rich, misfolded version of the host-encoded prion protein (PrPC), known as PrPSc. PrPSc self-replicates through a template-assisted process that converts the α-helical conformation of PrPC into the disease-associated isoform. In parallel with PrPSc accumulation, spongiform change is pathologically observed in the central nervous system, where “holes” appear because of massive neuronal death. Here, we review the cellular pathways triggered in response to PrPSc formation and accumulation. Available data suggest that neuronal dysfunction and death may be caused by what originates as a cellular pro-survival response to chronic PrPSc accumulation. We also discuss what is known about the complex cross-talk between the endoplasmic reticulum stress components and the quality control pathways. Better knowledge about these processes may lead to innovative therapeutic strategies based on manipulating the stress response and its consequences for neurodegeneration.This article is part of a Special Issue entitled SI:ER stress.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Brain Research - Volume 1648, Part B, 1 October 2016, Pages 553-560
نویسندگان
, ,