Targeting Cellular Prion Protein Reverses Early Cognitive Deficits and Neurophysiological Dysfunction in Prion-Infected Mice

• Published in: Neuron (Cambridge, Mass.) Vol. 53; no. 3; pp. 325 - 335
• Main Authors: Mallucci, Giovanna R., White, Melanie D., Farmer, Michael, Dickinson, Andrew, Khatun, Husna, Powell, Andrew D., Brandner, Sebastian, Jefferys, John G.R., Collinge, John
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.02.2007; Elsevier Limited
• Subjects: Animals; Axons - physiology; Behavior; Behavior, Animal - physiology; Brain - pathology; Brain research; Cognition Disorders - prevention & control; Cognition Disorders - psychology; Discrimination, Psychological - physiology; Electrophysiology; Hippocampus - pathology; HUMDISEASE; Immunohistochemistry; Infections; Long-Term Potentiation - physiology; Memory; Memory Disorders - etiology; Memory Disorders - psychology; Mice; Mice, Transgenic; Motor Activity - physiology; Muscle, Skeletal - physiology; Nesting Behavior - physiology; Pathology; Prion Diseases - genetics; Prion Diseases - pathology; Prion Diseases - psychology; Prions; PrPC Proteins - genetics; Psychomotor Performance - physiology; Reverse Transcriptase Polymerase Chain Reaction; Synapses - pathology; Synapses - physiology; SYSNEURO; Visual Perception - physiology; SYSNEURO; HUMDISEASE

Currently, no treatment can prevent the cognitive and motor decline associated with widespread neurodegeneration in prion disease. However, we previously showed that targeting endogenous neuronal prion protein (PrP C) (the precursor of its disease-associated isoform, PrP Sc) in mice with early prion...