Small heat shock proteins in neurodegenerative diseases

Cell Stress Chaperones. 2020 Jul;25(4):679-699. doi: 10.1007/s12192-020-01101-4. Epub 2020 Apr 22.

Abstract

Small heat shock proteins are ubiquitously expressed chaperones, yet mutations in some of them cause tissue-specific diseases. Here, we will discuss how small heat shock proteins give rise to neurodegenerative disorders themselves while we will also highlight how these proteins can fulfil protective functions in neurodegenerative disorders caused by protein aggregation. The first half of this paper will be focused on how mutations in HSPB1, HSPB3, and HSPB8 are linked to inherited peripheral neuropathies like Charcot-Marie-Tooth (CMT) disease and distal hereditary motor neuropathy (dHMN). The second part of the paper will discuss how small heat shock proteins are linked to neurodegenerative disorders like Alzheimer's, Parkinson's, and Huntington's disease.

Keywords: Diseases of the central nervous system; Hereditary peripheral neuropathies; Neurodegeneration; Protein aggregation; Small heat shock proteins.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Alzheimer Disease* / genetics
  • Alzheimer Disease* / metabolism
  • Charcot-Marie-Tooth Disease* / genetics
  • Charcot-Marie-Tooth Disease* / metabolism
  • Heat-Shock Proteins / genetics
  • Heat-Shock Proteins, Small / genetics*
  • Humans
  • Huntington Disease* / genetics
  • Huntington Disease* / metabolism
  • Molecular Chaperones / genetics
  • Mutation
  • Parkinson Disease* / genetics
  • Parkinson Disease* / metabolism

Substances

  • HSPB1 protein, human
  • HSPB3 protein, human
  • HSPB8 protein, human
  • Heat-Shock Proteins
  • Heat-Shock Proteins, Small
  • Molecular Chaperones