Mitochondrial chaperones in human health and disease

Free Radic Biol Med. 2022 Feb 1:179:363-374. doi: 10.1016/j.freeradbiomed.2021.11.015. Epub 2021 Nov 12.

Abstract

Molecular chaperones are a family of proteins that maintain cellular protein homeostasis through non-covalent peptide folding and quality control mechanisms. The chaperone proteins found within mitochondria play significant protective roles in mitochondrial biogenesis, quality control, and stress response mechanisms. Defective mitochondrial chaperones have been implicated in aging, neurodegeneration, and cancer. In this review, we focus on the two most prominent mitochondrial chaperones: mtHsp60 and mtHsp70. These proteins demonstrate different cellular localization patterns, interact with different targets, and have different functional activities. We discuss the structure and function of these prominent mitochondrial chaperone proteins and give an update on newly discovered regulatory mechanisms and disease implications.

Keywords: Mitochondrial chaperone; Mitochondrial homeostasis; Stressresponse; mtHsp60; mtHsp70.

Publication types

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

MeSH terms

  • HSP70 Heat-Shock Proteins* / metabolism
  • Humans
  • Mitochondria / metabolism
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism
  • Molecular Chaperones* / genetics
  • Molecular Chaperones* / metabolism
  • Protein Folding

Substances

  • HSP70 Heat-Shock Proteins
  • Mitochondrial Proteins
  • Molecular Chaperones