Doppel-induced apoptosis and counteraction by cellular prion protein in neuroblastoma and astrocytes

Neuroscience. 2006 Sep 1;141(3):1375-88. doi: 10.1016/j.neuroscience.2006.04.068. Epub 2006 Jun 12.

Abstract

Expression of a prion-like protein, doppel, induces apoptosis-like changes in cerebellar neuronal granule and Purkinje cells of prion-knockout mice and this effect can be rescued by re-introduction of cellular prion. Since most of those studies were done in transgenic mice, in the present study, we have established a murine neuro-2a cell line and the primary rat adult reactive astrocyte model for studying doppel-induced apoptosis and possible prion counteraction. We demonstrate that expression of doppel in neuro-2a cells causes apoptosis, during which DNA fragmentation occurs as visualized by terminal deoxynucleotidyl transferase biotin-dUTP nick end labeling staining and other intracellular changes characteristic of apoptosis are observed in the electron microscope. Using immunoblot analyses, we further demonstrate that doppel expression activates caspase-10 as well as caspase-3, but does not activate caspase-9. Addition of purified doppel to cultures of neuro-2a cells and the primary astrocytes causes similar apoptotic changes. Significantly, apoptosis induced by doppel is enhanced when cellular prion protein is depleted by RNA interference, suggesting a protective effect of cellular prion against doppel-induced apoptosis. The antagonistic interaction between cellular prion and doppel appears to involve direct protein-protein interaction possibly on cell membrane as cellular prion and doppel physically interact with each other and co-localize on cell membranes. Together, our data show that doppel induces apoptosis in neuroblastoma neuro-2a and rat primary astrocytes via a caspase-10 mediated pathway and that this effect is counteracted by cellular prion through direct interaction with doppel possibly on cell membrane.

Publication types

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

MeSH terms

  • Animals
  • Apoptosis / drug effects*
  • Astrocytes / drug effects*
  • Astrocytes / ultrastructure
  • Blotting, Western / methods
  • Caspases / metabolism
  • Cells, Cultured
  • Drug Interactions
  • Fluorescent Antibody Technique / methods
  • GPI-Linked Proteins
  • Gene Expression / drug effects
  • Gene Expression / physiology
  • In Situ Nick-End Labeling / methods
  • Indoles
  • Mice
  • Microscopy, Electron, Transmission / methods
  • Neuroblastoma / metabolism
  • Neuroblastoma / pathology*
  • Neuroblastoma / ultrastructure
  • Prions / metabolism
  • Prions / pharmacology
  • Prions / physiology*
  • RNA Interference / physiology
  • Rats
  • Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization / methods
  • Time Factors
  • Transfection / methods

Substances

  • GPI-Linked Proteins
  • Indoles
  • Prions
  • Prnd protein, mouse
  • DAPI
  • Caspases