Intracerebroventricular delivery of dominant negative prion protein in a mouse model of iatrogenic Creutzfeldt-Jakob disease after dura graft transplantation

Neurosci Lett. 2006 Jul 24;402(3):222-6. doi: 10.1016/j.neulet.2006.03.062. Epub 2006 Jun 6.

Abstract

We have developed a novel procedure in which a small collagen sheet (3 mm x 3 mm) absorbing prion-infected brain homogenates was transplanted onto the brain surface of highly prion-susceptible transgenic mice (Tg(MoPrP)4053/FVB), as an animal model of iatrogenic Creutzfeldt-Jakob disease (iCJD) caused by prion-contaminated cadaveric dura graft transplantation. Using the iCJD model, we further investigated the in vivo efficacy of dominant negative recombinant prion protein with lysine substitution at mouse codon 218 (rPrP-Q218K), which is known to inhibit prion replication in vitro (H. Kishida, Y. Sakasegawa, K. Watanabe, Y. Yamakawa, M. Nishijima, Y. Kuroiwa, N.S. Hachiya, K. Kaneko, Non-glycosylphosphatidylinositol (GPI)-anchored recombinant prion protein with dominant-negative mutation inhibits PrPSc replication in vitro, Amyloid, vol. 11, 2004, pp. 14-20.). Following 7-day intracerebroventricular administration of the rPrP-Q218K via an indwelling catheter connected to the implanted osmotic pump, the median incubation period of Tg(MoPrP)4053/FVB was prolonged considerably from 117 days to 131 days (p=0.016, log-rank test) in the rPrP-Q218K-treated group, even after a lengthy latency period of as long as 30 days by starting the rPrP-Q218K injection. Whether wild-type rPrP, other mutant rPrPs, or the combination of rPrP-Q218K with other anti-prion compounds might extend the survival period in that condition must be further investigated.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Catheters, Indwelling
  • Collagen
  • Creutzfeldt-Jakob Syndrome / metabolism*
  • Creutzfeldt-Jakob Syndrome / prevention & control
  • Creutzfeldt-Jakob Syndrome / transmission
  • Disease Models, Animal*
  • Dura Mater / transplantation*
  • Genes, Dominant
  • Iatrogenic Disease*
  • Infusion Pumps
  • Injections, Intraventricular
  • Mice
  • Mice, Transgenic
  • Mutation
  • Osmosis
  • PrPC Proteins / antagonists & inhibitors
  • Prions / administration & dosage*
  • Prions / genetics
  • Recombinant Proteins / administration & dosage
  • Recombinant Proteins / genetics

Substances

  • PrPC Proteins
  • Prions
  • Recombinant Proteins
  • Collagen