Disaggregases, molecular chaperones that resolubilize protein aggregates

An Acad Bras Cienc. 2015 Aug;87(2 Suppl):1273-92. doi: 10.1590/0001-3765201520140671. Epub 2015 Aug 25.

Abstract

The process of folding is a seminal event in the life of a protein, as it is essential for proper protein function and therefore cell physiology. Inappropriate folding, or misfolding, can not only lead to loss of function, but also to the formation of protein aggregates, an insoluble association of polypeptides that harm cell physiology, either by themselves or in the process of formation. Several biological processes have evolved to prevent and eliminate the existence of non-functional and amyloidogenic aggregates, as they are associated with several human pathologies. Molecular chaperones and heat shock proteins are specialized in controlling the quality of the proteins in the cell, specifically by aiding proper folding, and dissolution and clearance of already formed protein aggregates. The latter is a function of disaggregases, mainly represented by the ClpB/Hsp104 subfamily of molecular chaperones, that are ubiquitous in all organisms but, surprisingly, have no orthologs in the cytosol of metazoan cells. This review aims to describe the characteristics of disaggregases and to discuss the function of yeast Hsp104, a disaggregase that is also involved in prion propagation and inheritance.

Publication types

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

MeSH terms

  • Amyloid / biosynthesis
  • Amyloid / chemistry
  • Endopeptidase Clp
  • Escherichia coli Proteins / genetics*
  • Escherichia coli Proteins / metabolism
  • Heat-Shock Proteins / genetics*
  • Heat-Shock Proteins / metabolism
  • Heat-Shock Proteins / physiology*
  • Humans
  • Molecular Chaperones / genetics
  • Molecular Chaperones / physiology*
  • Prions / genetics*
  • Prions / metabolism
  • Protein Aggregates / genetics
  • Protein Aggregates / physiology*
  • Protein Folding*
  • Saccharomyces cerevisiae Proteins / genetics
  • Saccharomyces cerevisiae Proteins / physiology*
  • Substrate Specificity / genetics

Substances

  • Amyloid
  • Escherichia coli Proteins
  • Heat-Shock Proteins
  • Molecular Chaperones
  • Prions
  • Protein Aggregates
  • Saccharomyces cerevisiae Proteins
  • HsP104 protein, S cerevisiae
  • Endopeptidase Clp
  • ClpB protein, E coli