Báo cáo khoa học: Prions and prion diseases

Prions and Prion Diseases

Author: Michael Beekes

Field: Neurodegenerative Diseases / Prion Biology

Document Content:
This minireview series examines transmissible spongiform encephalopathies (TSEs), also known as prion diseases, a group of neurodegenerative disorders affecting the central nervous system. These diseases are characterized by the accumulation of an abnormally folded prion protein (PrPTSE) in the CNS. The series discusses the controversial nature of TSE agents, the prion hypothesis suggesting they are composed of misfolded prion protein, and recent advances in in vitro generation of infectious prions. It also highlights the transmission routes of TSEs, particularly peroral infections, and the ongoing need for research into their pathogenesis and the molecular mechanisms of prion spread. Furthermore, the review touches upon the neuropathological features of TSEs, the unknown molecular pathways leading to neurodegeneration, and the current lack of effective prophylactics or therapeutics, drawing parallels with other protein-misfolding disorders like Alzheimer’s and Parkinson’s disease.

Detailed Table of Contents:

• Prions and prion diseases
• The epidemic of bovine spongiform encephalopathy (BSE), or ‘mad cow disease’, and the subsequent emergence of a new variant of Creutzfeldt-Jakob disease (vCJD) in humans, has directed great political and scientific attention to a family of related neurodegenerative protein-misfolding diseases, collectively known as transmissible spongiform encephalopathies (TSEs) or prion diseases.
• The puzzling properties of scrapie- and other TSE agents have caused a vivid controversial debate about the molecular nature and biochemical composition of these pathogens for many years.
• Prions underlie the transmission of TSEs in the animal kingdom, between humans, and from animals to humans.
• Apart from the deposition of PrPTSE, features of CNS neuropathology in scrapie and other TSEs often include vacuolar (or ‘spongiform’) change, glial activation, synaptic degeneration and loss of neurons.
• To date, no effective prophylactics or therapeutics against TSEs are available.