Endoplasmic reticulum stress and prion diseases.

The endoplasmic reticulum (ER), an important cellular organelle in eukaryotic cells, becomes dysfunctional following exposure to harmful stimuli. These stimuli can cause the ER stress response, which induces cell apoptosis due to changes in ER protein levels such as glucose-regulated protein. Current studies indicate that ER stress is closely related to the occurrence and development of neurodegenerative disorders, e.g., prion diseases. The pathogenic agent known as the misfolded prion protein may cause an imbalance in ER homeostasis and commit the neuron to a pathway of apoptosis; however, the specific mechanisms are still under intensive investigation. This review summarizes current research investigating the relationship between ER stress and prion diseases. These findings will aid in the development of novel strategies for diagnosis and therapies for prion and other neurodegenerative diseases.