Prion proteins from susceptible and resistant sheep exhibit some distinct cell biological features.
It is well established that natural polymorphisms in the coding sequence of the PrP protein can control the expression of prion disease. Studies with a cell model of sheep prion infection have shown that ovine PrP allele associated with resistance to sheep scrapie may confer resistance by impairing the multiplication of the infectious agent. To further explore the biochemical and cellular mechanisms underlying the genetic control of scrapie susceptibility, we established permissive cells expressing two different PrP variants. In this study, we show that PrP variants with opposite effects on prion multiplication exhibit distinct cell biological features. These findings indicate that cell biological properties of ovine PrP can vary with natural polymorphisms and raise the possibility that differential interactions of PrP variants with the cellular machinery may contribute to permissiveness or resistance to prion multiplication.
Sabuncu
Elifsu
E
Unité de Virologie et Immunologie Moléculaires, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France.
Paquet
Sophie
S
Chapuis
Jérome
J
Moudjou
Mohammed
M
Lai
Thanh Lan
TL
Grassi
Jacques
J
Baron
Udo
U
Laude
Hubert
H
Vilette
Didier
D
D000595:Amino Acid Sequence; D000818:Animals; D002478:Cells, Cultured; D004198:Disease Susceptibility / Q000503:physiopathology*; D004847:Epithelial Cells / Q000737:chemistry* / Q000378:metabolism* / Q000473:pathology; D007113:Immunity, Innate / Q000502:physiology*; D008969:Molecular Sequence Data; D011328:Prions / Q000032:analysis / Q000737:chemistry* / Q000378:metabolism*; D021381:Protein Transport; D012756:Sheep; D013329:Structure-Activity Relationship
D011328:Prions