New Frontiers in Animal Prion Diseases

The transmissible spongiform encephalopathies are a group of fatal, progressive neurodegenerative disorders caused by the misfolding of prion proteins, leading to severe neuropathology and death. Since the description of scrapie in sheep several centuries ago, significant advancements have been made in understanding the spectrum of prion diseases, including bovine spongiform encephalopathy and Creutzfeldt–Jakob disease. Despite decades of research, critical gaps remain in our understanding of prion replication mechanisms, interspecies transmission, and the environmental persistence of prions. Advances in molecular imaging, including cryo-electron microscopy, have been instrumental in visualizing prion-associated aggregates in affected brain tissues, providing critical insights into their conformation and strain-specific structures. We explore the development of transmissible spongiform encephalopathy research in animals, major scientific breakthroughs, and the pressing need for innovative diagnostic and therapeutic approaches. Addressing these challenges is essential for controlling the spread of prion diseases, and reducing their impact on public health and agriculture.