NR AGFO
AU Katamine,S.; Nishida,N.; Sugimoto,T.; Noda,T.; Sakaguchi,S.; Shigematsu,K.; Kataoka,Y.; Nakatani,A.; Hasegawa,S.; Moriuchi,R.; Miyamoto,T.
TI Impaired motor coordination in mice lacking prion protein
QU Cellular and Molecular Neurobiology 1998 Dec; 18(6): 731-42
PT journal article; review; review, tutorial
AB 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.
ZR 56
MH Animal; Brain/pathology/*physiopathology; Cerebellum/physiology/physiopathology; Human; Mice; Mice, Knockout; Motor Activity/genetics/*physiology; Movement Disorders/*genetics/physiopathology; PrPc Proteins/genetics/*physiology; Prion Diseases/genetics/*physiopathology; Prions/genetics/*physiology; Purkinje Cells/physiology
AD Department of Bacteriology, Nagasaki University School of Medicine, Japan.
SP englisch
PO USA