Assistance for Folding of Disease-Causing Plasma Membrane Proteins

Biomolecules. 2020 May 7;10(5):728. doi: 10.3390/biom10050728.

Abstract

An extensive catalog of plasma membrane (PM) protein mutations related to phenotypic diseases is associated with incorrect protein folding and/or localization. These impairments, in addition to dysfunction, frequently promote protein aggregation, which can be detrimental to cells. Here, we review PM protein processing, from protein synthesis in the endoplasmic reticulum to delivery to the PM, stressing the main repercussions of processing failures and their physiological consequences in pathologies, and we summarize the recent proposed therapeutic strategies to rescue misassembled proteins through different types of chaperones and/or small molecule drugs that safeguard protein quality control and regulate proteostasis.

Keywords: chaperones; misrouting; proteostasis; quality control.

Publication types

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

MeSH terms

  • Animals
  • Channelopathies / drug therapy
  • Channelopathies / genetics
  • Channelopathies / metabolism*
  • Humans
  • Membrane Proteins / chemistry
  • Membrane Proteins / metabolism*
  • Molecular Chaperones / metabolism*
  • Protein Folding*
  • Protein Transport
  • Proteostasis Deficiencies / drug therapy
  • Proteostasis Deficiencies / genetics
  • Proteostasis Deficiencies / metabolism*

Substances

  • Membrane Proteins
  • Molecular Chaperones