title: The prion protein gene is dispensable for the development of spongiform myeloencephalopathy induced by the neurovirulent Cas-Br-E murine leukemia virus.
abstract: The Cas-Br-E murine leukemia virus (MuLV) induces paralysis in susceptible mice that is accompanied by a severe spongiform myeloencephalopathy. These neurodegenerative lesions are very similar to those observed in prion diseases. To determine whether the prion protein gene (Prn-p) product was a downstream effector of this neurovirulent MuLV, we inoculated Prn-p(-/-) knockout homozygote and control heterozygote or wild-type mice with this retrovirus. All groups developed typical paralysis and spongiform encephalopathy, and no differences in clinical or histological phenotypes were observed between these groups. These results indicate that the Cas-Br-E MuLV does not require the prion protein to induce lesions. Thus, MuLV and prion proteins may induce a very similar disease through distinct pathways, or the viral Env protein, which harbors the primary determinant of pathogenicity, may act in a common pathway but downstream of the prion protein.
authors.affiliation: Laboratory of Molecular Biology, Clinical Research Institute of Montreal, Québec, Canada. Jolicop@IRCM.UMontreal.CA
authors.forename: P
authors.initials: P
authors.lastname: Jolicoeur
authors.forename: G
authors.initials: G
authors.lastname: Massé
authors.forename: D G
authors.initials: DG
authors.lastname: Kay
mesh_terms: D000818:Animals; D002490:Central Nervous System / Q000473:pathology / Q000821:virology; D005260:Female; D017353:Gene Deletion; D009052:Leukemia Virus, Murine / Q000302:isolation & purification / Q000472:pathogenicity*; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D009410:Nerve Degeneration; D010243:Paralysis / Q000378:metabolism* / Q000473:pathology / Q000821:virology; D018621:PrPC Proteins / Q000235:genetics / Q000378:metabolism*; D017096:Prion Diseases / Q000378:metabolism* / Q000473:pathology / Q000821:virology; D014774:Virulence
chemical_list: D018621:PrPC Proteins