Protein and lipid interactions - Modulating CFTR trafficking and rescue

J Cyst Fibros. 2018 Mar;17(2S):S9-S13. doi: 10.1016/j.jcf.2017.08.014. Epub 2017 Sep 5.

Abstract

Different levels of CFTR regulation in the cell contribute to a stringent control of chloride secretion in epithelia. Tuning of chloride transport is achieved by modulating CFTR biogenesis, exit from the endoplasmic reticulum, trafficking, membrane stability and channel activity. In this short review, we summarize recent findings identifying interactions with other proteins - directly or through membrane lipids - and briefly discuss how these observations can provide clues to the design of better therapeutic approaches.

Keywords: Endoplasmic reticulum; Lipids; Membrane stability; Sphingomyelin; cAMP.

Publication types

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

MeSH terms

  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis Transmembrane Conductance Regulator* / chemistry
  • Cystic Fibrosis Transmembrane Conductance Regulator* / metabolism
  • Endoplasmic Reticulum / physiology
  • Humans
  • Ion Transport

Substances

  • Cystic Fibrosis Transmembrane Conductance Regulator