title: Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits.
abstract: Atypical bovine spongiform encephalopathy (BSE) has recently been identified in Europe, North America, and Japan. It is classified as H-type and L-type BSE according to the molecular mass of the disease-associated prion protein (Pr(PSc)). To investigate the topographical distribution and deposition patterns of immunolabeled Pr(PSc), H-type BSE isolate was inoculated intracerebrally into cattle. H-type BSE was successfully transmitted to 3 calves, with incubation periods between 500 and 600 days. Moderate to severe spongiform changes were detected in the cerebral and cerebellar cortices, basal ganglia, thalamus, and brainstem. H-type BSE was characterized by the presence of PrP-immunopositive amyloid plaques in the white matter of the cerebrum, basal ganglia, and thalamus. Moreover, intraglial-type immunolabeled Pr(PSc) was prominent throughout the brain. Stellate-type immunolabeled Pr(PSc) was conspicuous in the gray matter of the cerebral cortex, basal ganglia, and thalamus, but not in the brainstem. In addition, Pr(PSc) accumulation was detected in the peripheral nervous tissues, such as trigeminal ganglia, dorsal root ganglia, optic nerve, retina, and neurohypophysis. Cattle are susceptible to H-type BSE with a shorter incubation period, showing distinct and distinguishable phenotypes of Pr(PSc) accumulation.
authors.affiliation: Prion Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba, Ibaraki 305-0856, Japan. okadahi@affrc.go.jp.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Okada
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Iwamaru
authors.forename: Morikazu
authors.initials: M
authors.lastname: Imamura
authors.forename: Kentaro
authors.initials: K
authors.lastname: Masujin
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Matsuura
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Shimizu
authors.forename: Kazuo
authors.initials: K
authors.lastname: Kasai
authors.forename: Shirou
authors.initials: S
authors.lastname: Mohri
authors.forename: Takashi
authors.initials: T
authors.lastname: Yokoyama
authors.forename: Stefanie
authors.initials: S
authors.lastname: Czub
mesh_terms: D000818:Animals; D015153:Blotting, Western / Q000662:veterinary; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000209:etiology / Q000378:metabolism* / Q000635:transmission*; D008859:Microscopy, Polarization / Q000662:veterinary; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction / Q000662:veterinary; D018620:PrPSc Proteins / Q000378:metabolism*; D017422:Sequence Analysis, DNA / Q000662:veterinary
chemical_list: D018620:PrPSc Proteins