Bimodal protein targeting through activation of cryptic mitochondrial targeting signals by an inducible cytosolic endoprotease

Mol Cell. 2008 Oct 10;32(1):32-42. doi: 10.1016/j.molcel.2008.09.008.

Abstract

Bimodal targeting of the endoplasmic reticular protein, cytochrome P4501A1 (CYP1A1), to mitochondria involves activation of a cryptic mitochondrial targeting signal through endoprotease processing of the protein. Here, we characterized the endoprotease that regulates mitochondrial targeting of CYP1A1. The endoprotease, which was induced by beta-naphthoflavone, was a dimer of 90 kDa and 40 kDa subunits, each containing Ser protease domains. The purified protease processed CYP1A1 in a sequence-specific manner, leading to its mitochondrial import. The glucocorticoid receptor, retinoid X receptor, and p53 underwent similar processing-coupled mitochondrial transport. The inducible 90 kDa subunit was a limiting factor in many cells and some tissues and, thus, regulates the mitochondrial levels of these proteins. A number of other mitochondria-associated proteins with noncanonical targeting signals may also be substrates of this endoprotease. Our results describe a new mechanism of mitochondrial protein import that requires an inducible cytoplasmic endoprotease for activation of cryptic mitochondrial targeting signals.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Cell Line
  • Cytochrome P-450 CYP1A1 / metabolism
  • Humans
  • Male
  • Mice
  • Mitochondria / metabolism*
  • Molecular Weight
  • Peptide Hydrolases / chemistry
  • Peptide Hydrolases / genetics
  • Peptide Hydrolases / metabolism*
  • Protein Subunits
  • Protein Transport
  • RNA, Small Interfering / genetics
  • Rats
  • Rats, Sprague-Dawley
  • Signal Transduction
  • Substrate Specificity
  • beta-Naphthoflavone / pharmacology

Substances

  • Protein Subunits
  • RNA, Small Interfering
  • beta-Naphthoflavone
  • Cytochrome P-450 CYP1A1
  • Peptide Hydrolases