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Publication : Impaired motor coordination in mice lacking prion protein.

First Author  Katamine S Year  1998
Journal  Cell Mol Neurobiol Volume  18
Issue  6 Pages  731-42
PubMed ID  9876879 Mgi Jnum  J:52329
Mgi Id  MGI:1328870 Doi  10.1023/a:1020698305911
Citation  Katamine S, et al. (1998) Impaired motor coordination in mice lacking prion protein. Cell Mol Neurobiol 18(6):731-42
abstractText  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.
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