title: Biological and biochemical characteristics of prion strains conserved in persistently infected cell cultures.
abstract: Abnormal prion protein (PrP(Sc)) plays a central role in the transmission of prion diseases, but the molecular basis of prion strains with distinct biological characteristics remains to be elucidated. We analyzed the characteristics of prion disease by using mice inoculated with the Chandler and Fukuoka-1 strains propagated in a cultured mouse neuronal cell line, GT1-7, which is highly permissive to replication of the infectious agents. Strain-specific biological characteristics, including clinical manifestations, incubation period as related to the infectious unit, and pathological profiles, remained unchanged after passages in the cell cultures. We noted some differences in the biochemical aspects of PrP(Sc) between brain tissues and GT1-7 cells which were unlikely to affect the strain phenotypes. On the other hand, the proteinase K-resistant PrP core fragments derived from Fukuoka-1-infected tissues and cells were slightly larger than those from Chandler-infected versions. Moreover, Fukuoka-1 infection, but not Chandler infection, gave an extra fragment with a low molecular weight, approximately 13 kDa, in both brain tissues and GT1-7 cells. This cell culture model persistently infected with different strains will provide a new insight into the understanding of the molecular basis of prion diversity.
authors.affiliation: Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Sakamoto 1-12-4, Nagasaki 852-8523, Japan.
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Arima
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Nishida
authors.forename: Suehiro
authors.initials: S
authors.lastname: Sakaguchi
authors.forename: Kazuto
authors.initials: K
authors.lastname: Shigematsu
authors.forename: Ryuichiro
authors.initials: R
authors.lastname: Atarashi
authors.forename: Naohiro
authors.initials: N
authors.lastname: Yamaguchi
authors.forename: Daisuke
authors.initials: D
authors.lastname: Yoshikawa
authors.forename: Jaewoo
authors.initials: J
authors.lastname: Yoon
authors.forename: Ken
authors.initials: K
authors.lastname: Watanabe
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Kobayashi
authors.forename: Sophie
authors.initials: S
authors.lastname: Mouillet-Richard
authors.forename: Sylvain
authors.initials: S
authors.lastname: Lehmann
authors.forename: Shigeru
authors.initials: S
authors.lastname: Katamine
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D002460:Cell Line; D006801:Humans; D051379:Mice; D009474:Neurons / Q000737:chemistry; D018620:PrPSc Proteins / Q000737:chemistry / Q000145:classification / Q000472:pathogenicity; D017096:Prion Diseases / Q000209:etiology / Q000473:pathology / Q000635:transmission; D011328:Prions / Q000737:chemistry* / Q000145:classification / Q000472:pathogenicity*; D013045:Species Specificity
chemical_list: D018620:PrPSc Proteins; D011328:Prions