Molecular chaperone dysfunction in neurodegenerative diseases and effects of curcumin

Biomed Res Int. 2014:2014:495091. doi: 10.1155/2014/495091. Epub 2014 Oct 19.

Abstract

The intra- and extracellular accumulation of misfolded and aggregated amyloid proteins is a common feature in several neurodegenerative diseases, which is thought to play a major role in disease severity and progression. The principal machineries maintaining proteostasis are the ubiquitin proteasomal and lysosomal autophagy systems, where heat shock proteins play a crucial role. Many protein aggregates are degraded by the lysosomes, depending on aggregate size, peptide sequence, and degree of misfolding, while others are selectively tagged for removal by heat shock proteins and degraded by either the proteasome or phagosomes. These systems are compromised in different neurodegenerative diseases. Therefore, developing novel targets and classes of therapeutic drugs, which can reduce aggregates and maintain proteostasis in the brains of neurodegenerative models, is vital. Natural products that can modulate heat shock proteins/proteosomal pathway are considered promising for treating neurodegenerative diseases. Here we discuss the current knowledge on the role of HSPs in protein misfolding diseases and knowledge gained from animal models of Alzheimer's disease, tauopathies, and Huntington's diseases. Further, we discuss the emerging treatment regimens for these diseases using natural products, like curcumin, which can augment expression or function of heat shock proteins in the cell.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Alzheimer Disease / drug therapy
  • Alzheimer Disease / metabolism*
  • Alzheimer Disease / pathology
  • Curcumin / therapeutic use*
  • Heat-Shock Proteins / metabolism
  • Humans
  • Huntington Disease / drug therapy
  • Huntington Disease / metabolism*
  • Huntington Disease / pathology
  • Molecular Chaperones / metabolism
  • Neurodegenerative Diseases / drug therapy
  • Neurodegenerative Diseases / metabolism*
  • Neurodegenerative Diseases / pathology
  • Protein Aggregation, Pathological / metabolism
  • Protein Folding
  • Proteostasis Deficiencies / drug therapy
  • Proteostasis Deficiencies / metabolism
  • Proteostasis Deficiencies / pathology
  • Ubiquitin / genetics
  • Ubiquitin / metabolism

Substances

  • Heat-Shock Proteins
  • Molecular Chaperones
  • Ubiquitin
  • Curcumin