Endoplasmic reticulum stress, PrP trafficking, and neurodegeneration.
In this issue of Developmental Cell, Rane et al. report a cellular pathway to link PrP(Sc), via ER stress and the activation of a preemptive quality control process, to neurodegeneration in a PrP-dependent manner. This pathway puts together several pieces in the puzzle of the relationship between PrP(Sc) and brain damage and may in part explain the mechanism of prion neurodegeneration.
Soto
Claudio
C
George and Cynthia Mitchell Center for Neurodegenerative Diseases, Departments of Neurology, Neuroscience, and Cell Biology and Biochemistry and Molecular Biology, University of Texas Medical Branch, Galveston, TX 77555, USA. Electronic address: clsoto@utmb.edu.
D000818:Animals; D004721:Endoplasmic Reticulum / Q000378:metabolism*; D051379:Mice; D008954:Models, Biological; D009410:Nerve Degeneration / Q000378:metabolism* / Q000473:pathology; D018384:Oxidative Stress; D018620:PrPSc Proteins / Q000378:metabolism*; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism; D021381:Protein Transport / Q000502:physiology
D018620:PrPSc Proteins; D046988:Proteasome Endopeptidase Complex