Affiliation:
1. Department of Pharmaceutical Sciences,Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.),,Lucknow,India,226025,
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.
Publisher
BENTHAM SCIENCE PUBLISHERS