title: Impaired motor coordination in mice lacking prion protein.
abstract: 1. Prion protein (PrPC) is a host-encoded glycoprotein constitutively expressed on the neuronal cell surface. Accumulation of its protease-resistant isoform is closely related to pathologic changes and prion propagation in the brain tissue of a series of prion diseases. However, the physiological role of PrPC remains to be elucidated. 2. After long-term observation, we noted impaired motor coordination and loss of cerebellar Purkinje cells in the aged mice homozygous for a disrupted PrP gene, a finding which strongly suggests that PrPC plays a role in the long-term survival of Purkinje cells. 3. We also describe the resistance of the PrP null mice to the prion, indicating the requirement of PrPC for both the development of prion diseases and the prion propagation.
authors.affiliation: Department of Bacteriology, Nagasaki University School of Medicine, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Katamine
authors.forename: N
authors.initials: N
authors.lastname: Nishida
authors.forename: T
authors.initials: T
authors.lastname: Sugimoto
authors.forename: T
authors.initials: T
authors.lastname: Noda
authors.forename: S
authors.initials: S
authors.lastname: Sakaguchi
authors.forename: K
authors.initials: K
authors.lastname: Shigematsu
authors.forename: Y
authors.initials: Y
authors.lastname: Kataoka
authors.forename: A
authors.initials: A
authors.lastname: Nakatani
authors.forename: S
authors.initials: S
authors.lastname: Hasegawa
authors.forename: R
authors.initials: R
authors.lastname: Moriuchi
authors.forename: T
authors.initials: T
authors.lastname: Miyamoto
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D002531:Cerebellum / Q000502:physiology / Q000503:physiopathology; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D009043:Motor Activity / Q000235:genetics / Q000502:physiology*; D009069:Movement Disorders / Q000235:genetics* / Q000503:physiopathology; D018621:PrPC Proteins / Q000235:genetics / Q000502:physiology*; D017096:Prion Diseases / Q000235:genetics / Q000503:physiopathology*; D011328:Prions / Q000235:genetics / Q000502:physiology*; D011689:Purkinje Cells / Q000502:physiology
chemical_list: D018621:PrPC Proteins; D011328:Prions