The intricate mechanisms of neurodegeneration in prion diseases.
Prion diseases are a group of infectious neurodegenerative diseases with an entirely novel mechanism of transmission, involving a protein-only infectious agent that propagates the disease by transmitting protein conformational changes. The disease results from extensive and progressive brain degeneration. The molecular mechanisms involved in neurodegeneration are not entirely known but involve multiple processes operating simultaneously and synergistically in the brain, including spongiform degeneration, synaptic alterations, brain inflammation, neuronal death and the accumulation of protein aggregates. Here, we review the pathways implicated in prion-induced brain damage and put the pieces together into a possible model of neurodegeneration in prion disorders. A more comprehensive understanding of the molecular basis of brain degeneration is essential to develop a much needed therapy for these devastating diseases.
Soto
Claudio
C
Mitchell Center for Alzheimer's disease and related Brain disorders, Department of Neurology, University of Texas Houston Medical School, 6431 Fannin St, Houston, TX 77030, USA.
Satani
Nikunj
N
D000818:Animals; D001921:Brain; D006801:Humans; D009410:Nerve Degeneration; D009474:Neurons / Q000166:cytology / Q000378:metabolism; D017096:Prion Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D011328:Prions / Q000378:metabolism
D011328:Prions