Protein Misfolding, a Common Mechanism in the Pathogenesis of Neurodegenerative Diseases
Neurodegenerative diseases are some of the most debilitating disorders, affecting abstract thinking, skilled movements, emotional feelings, cognition, and memory. Recent and compelling evidence indicates that the misfolding, aggregation, and accumulation
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Protein Misfolding, a Common Mechanism in the Pathogenesis of Neurodegenerative Diseases
L. Vergara . K. Abid . C. Soto
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Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286
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Role of Protein Misfolding and Aggregation in Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287
3 3.1 3.2 3.3 3.4 3.5
Clinical, Neuropathological, and Molecular Features of Neurodegenerative Diseases . . . . . . . . . 288 Alzheimer’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 288 Parkinson Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 290 Huntington Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 290 Amyotrophic Lateral Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 291 Prion Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292
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Animal Models of Neurodegenerative Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293
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Structural and Mechanistic Basis of Protein Misfolding and Aggregation . . . . . . . . . . . . . . . . . . . . . 294
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Mechanism of Brain Degeneration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 296
7
Targets for Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 296
8
Conclusions and Perspectives . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298
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2008 Springer ScienceþBusiness Media, LLC.
286
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Protein misfolding, a common mechanism in the pathogenesis of neurodegenerative diseases
Abstract: Neurodegenerative diseases are some of the most debilitating disorders, affecting abstract thinking, skilled movements, emotional feelings, cognition, and memory. Recent and compelling evidence indicates that the misfolding, aggregation, and accumulation of proteins in the brain may be the cause of these diseases. The aim of this chapter is to review the literature around the molecular mechanism and role of misfolded protein aggregates in neurodegeneration and the possibilities for therapeutic intervention. List of Abbreviations: AD, Alzheimer’s disease; ALS, amyotrophic lateral sclerosis; APP, amyloi
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