Lipid-mediated, reversible misfolding of a sterol-sensing domain protein

EMBO J. 2005 Jan 12;24(1):149-59. doi: 10.1038/sj.emboj.7600498. Epub 2005 Jan 6.

Abstract

Cellular quality control requires recognition of common features of misfolding, and so is not typically associated with the specific targeting of individual proteins. However, physiologically regulated degradation of yeast HMG-CoA reductase (Hmg2p) occurs by the HRD endoplasmic reticulum quality control pathway, implying that Hmg2p undergoes a regulated transition to a quality control substrate in response to a sterol pathway molecule. Using in vitro structural assays, we now show that the pathway derivative farnesol causes Hmg2p to undergo a change to a less folded structure. The effect is reversible, biologically relevant by numerous criteria, highly specific for farnesol structure, and requires an intact Hmg2p sterol-sensing domain. This represents a distinct lipid-sensing function for this highly conserved motif that suggests novel approaches to cholesterol management. More generally, our observation of reversible small-molecule-mediated misfolding may herald numerous examples of regulated quality control to be discovered in biology or applied in the clinic.

Publication types

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

MeSH terms

  • Farnesol / chemistry*
  • Farnesol / metabolism*
  • Hydroxymethylglutaryl CoA Reductases / chemistry*
  • Hydroxymethylglutaryl CoA Reductases / genetics
  • Hydroxymethylglutaryl CoA Reductases / metabolism*
  • Lipid Metabolism*
  • Lipids / chemistry
  • Microsomes / metabolism
  • Molecular Structure
  • Protein Denaturation
  • Protein Folding*
  • Protein Structure, Secondary
  • Recombinant Fusion Proteins / chemistry
  • Recombinant Fusion Proteins / genetics
  • Recombinant Fusion Proteins / metabolism
  • Saccharomyces cerevisiae Proteins / chemistry
  • Saccharomyces cerevisiae Proteins / genetics
  • Saccharomyces cerevisiae Proteins / metabolism
  • Trypsin / metabolism

Substances

  • Lipids
  • Recombinant Fusion Proteins
  • Saccharomyces cerevisiae Proteins
  • Farnesol
  • Hydroxymethylglutaryl CoA Reductases
  • Trypsin