51. Mouse Models for Studying the Formation and Propagation of Prions*
- Author
-
Watts, Joel C and Prusiner, Stanley B
- Subjects
Biochemistry and Cell Biology ,Biomedical and Clinical Sciences ,Biological Sciences ,Infectious Diseases ,Emerging Infectious Diseases ,Rare Diseases ,Transmissible Spongiform Encephalopathy (TSE) ,Vaccine Related ,Neurosciences ,Neurodegenerative ,Brain Disorders ,Biodefense ,Prevention ,Underpinning research ,Aetiology ,2.1 Biological and endogenous factors ,1.1 Normal biological development and functioning ,Neurological ,Alzheimer Disease ,Amyloid beta-Peptides ,Animals ,Brain ,Disease Models ,Animal ,Humans ,Mice ,Mice ,Transgenic ,Mutation ,Parkinson Disease ,Prion Diseases ,Prions ,alpha-Synuclein ,tau Proteins ,Animal Model ,Neurodegenerative Disease ,Pathology ,Prion ,Protein Aggregation ,Chemical Sciences ,Medical and Health Sciences ,Biochemistry & Molecular Biology ,Biological sciences ,Biomedical and clinical sciences ,Chemical sciences - Abstract
Prions are self-propagating protein conformers that cause a variety of neurodegenerative disorders in humans and animals. Mouse models have played key roles in deciphering the biology of prions and in assessing candidate therapeutics. The development of transgenic mice that form prions spontaneously in the brain has advanced our understanding of sporadic and genetic prion diseases. Furthermore, the realization that many proteins can become prions has necessitated the development of mouse models for assessing the potential transmissibility of common neurodegenerative diseases. As the universe of prion diseases continues to expand, mouse models will remain crucial for interrogating these devastating illnesses.
- Published
- 2014