Helix 3 is necessary and sufficient for prion protein's anti-Bax function

J Neurochem. 2009 Feb;108(4):1019-31. doi: 10.1111/j.1471-4159.2008.05851.x.

Abstract

To identify the structural elements of the prion protein (PrP) necessary for its protective function against Bcl-2 associated protein X (Bax), we performed structure-function analyses of the anti-Bax function of cytosolic PrP (CyPrP) in MCF-7 cells. Deletions of 1, 2, or 3 N-terminal Bcl-2 homology domain 2-like octapeptide repeats (BORs), but not deletion of all four BORs, abolish CyPrPs anti-Bax function. Deletion of alpha-helix 3 (PrP23-199) or further C-terminal deletions of alpha-helix 1 and 2, and beta-strand 1 and 2 (PrP23-172, PrP23-160, PrP23-143, and PrP23-127) eliminates CyPrPs protection against Bax-mediated cell death. The substitution of helix 3 amino acid residues K204, V210, and E219 by proline inhibits the anti-Bax function of CyPrP. The substitution of K204, but not V210 and E219, by alanine residues also prevents CyPrPs anti-Bax function. Expression of PrPs helix 3 displays anti-Bax activity in MCF-7 cells and in human neurons. Together, these results indicate that although the BOR domain has an influence on PrPs anti-Bax function, the helix 3 is necessary and sufficient for the anti-Bax function of CyPrP. Identification of helix 3 as the structural element for the anti-Bax function thus provides a molecular target to modulate PrPs anti-Bax function in cancer and neurodegeneration.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alanine / chemistry
  • Amino Acid Sequence / physiology
  • Amino Acid Substitution / physiology
  • Animals
  • Apoptosis / physiology*
  • Cell Line, Tumor
  • Cells, Cultured
  • Cytoprotection / genetics
  • Humans
  • Mice
  • Neoplasms / genetics
  • Neoplasms / metabolism
  • Nerve Degeneration / genetics
  • Nerve Degeneration / metabolism
  • PrPC Proteins / chemistry*
  • PrPC Proteins / genetics
  • PrPC Proteins / metabolism
  • Prion Diseases / genetics
  • Prion Diseases / metabolism
  • Prion Diseases / physiopathology
  • Proline / chemistry
  • Protein Structure, Secondary / genetics
  • bcl-2-Associated X Protein / genetics
  • bcl-2-Associated X Protein / metabolism*

Substances

  • PrPC Proteins
  • bcl-2-Associated X Protein
  • Proline
  • Alanine