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.
Keywords: ALS, Alzheimer, Amyloid beta, Huntington, Misfolding, Parkinson, Protein aggregation, Protein folding.