Abstract

Protein aggregation-related diseases primarily affect the central nervous system and are involved in the pathogenesis of multiple neurodegenerative diseases as well as several rare hereditary disorders that involve the deposition of protein aggregates in the brain. These diseases include Alzheimer's, Parkinson, Huntington's disease, Prion diseases, amyotrophic lateral sclerosis, familial amyloid polyneuropathy, etc. The aggregates usually consist of fibers containing misfolded protein with a betasheet conformation. As a result, proteins’ secondary structures change from α-helix to β-sheet, leading to the accumulation of harmful misfolded protein aggregates in the CNS. The misfolding, subsequent aggregation and accumulation of proteins in neurodegenerative diseases lead to cellular dysfunction, loss of synaptic connections and brain damage. This chapter discusses some of the important neurodegenerative diseases resulting from protein misfolding and explains the pathological mechanisms behind brain damage.