Prion protein contains a second endoplasmic reticulum targeting signal sequence located at its C terminus

J Biol Chem. 2001 Apr 20;276(16):13388-94. doi: 10.1074/jbc.M007331200. Epub 2001 Jan 22.

Abstract

Prion protein (PrP) is synthesized at the membrane of the endoplasmic reticulum (ER) in three different topological forms as follows: a fully translocated one ((sec)PrP) and two with opposite orientations in the membrane ((Ntm)PrP and (Ctm)PrP). We asked whether other signal sequences exist in the PrP, other than the N-terminal signal sequence, that contribute to its topological diversity. In vitro translocation assays showed that PrP lacking its N-terminal signal sequence could still translocate into ER microsomes, although at reduced efficiency. Deletion of each of the two hydrophobic regions in PrP revealed that the C-terminally located hydrophobic region (TM2) can function as second signal sequence in PrP. Translocation mediated by the TM2 alone can occur post-translationally and yields mainly (Ctm)PrP, which is implicated in some forms of neurodegeneration in prion diseases. We conclude that, in vitro, PrP can insert into ER membranes co- and post-translationally and can use two different signal sequences. We propose that the unusually complex topology of PrP results from the differential utilization of two signal sequences in PrP.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Endopeptidases / metabolism
  • Endoplasmic Reticulum / metabolism*
  • Humans
  • Intracellular Membranes / metabolism*
  • Prion Diseases
  • Prions / chemistry*
  • Prions / genetics
  • Prions / metabolism*
  • Protein Biosynthesis
  • Protein Processing, Post-Translational
  • Protein Sorting Signals
  • RNA, Messenger / genetics
  • Recombinant Proteins / chemistry
  • Recombinant Proteins / metabolism
  • Sequence Deletion
  • Transcription, Genetic

Substances

  • Prions
  • Protein Sorting Signals
  • RNA, Messenger
  • Recombinant Proteins
  • Endopeptidases