title: The prion model for progression and diversity of neurodegenerative diseases.
abstract: The neuropathology of different neurodegenerative diseases begins in different brain regions, and involves distinct brain networks. Evidence indicates that transcellular propagation of protein aggregation, which is the basis of prion disease, might underlie the progression of pathology in neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and Huntington's disease. The prion model predicts specific patterns of neuronal vulnerability and network involvement on the basis of the conformation of pathological proteins. Indeed, evidence indicates that self-propagating aggregate conformers, or so-called strains, are associated with distinct neuropathological syndromes. The extension of this hypothesis to our understanding of common neurodegenerative disorders can suggest new therapeutic approaches, such as immunotherapy and small molecules, to block transcellular propagation, and new diagnostic tools to detect early evidence of disease.
authors.affiliation: Center for Alzheimer's and Neurodegenerative Diseases, Peter O'Donnell Jr Brain Institute, University of Texas, Southwestern Medical Center, Dallas, TX 75225, USA.
authors.forename: Barbara E
authors.initials: BE
authors.lastname: Stopschinski
authors.affiliation: Center for Alzheimer's and Neurodegenerative Diseases, Peter O'Donnell Jr Brain Institute, University of Texas, Southwestern Medical Center, Dallas, TX 75225, USA. Electronic address: marc.diamond@utsouthwestern.edu.
authors.forename: Marc I
authors.initials: MI
authors.lastname: Diamond
mesh_terms: D000818:Animals; D018450:Disease Progression; D006801:Humans; D008954:Models, Biological; D019636:Neurodegenerative Diseases / Q000145:classification / Q000473:pathology*; D017096:Prion Diseases / Q000473:pathology*