NR AIWW
AU Nishida,N.; Tremblay,P.; Sugimoto,T.; Shigematsu,K.; Shirabe,S.; Petromilli,C.; Erpel,S.P.; Nakaoke,R.; Atarashi,R.; Houtani,T.; Torchia,M.; Sakaguchi,S.; DeArmond,S.J.; Prusiner,S.B.; Katamine,S.
TI A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination
QU Laboratory Investigation 1999 Jun; 79(6): 689-97
PT journal article
AB Disruption of both alleles of the prion protein gene, Prnp, renders mice resistant to prions; in a Prnp o/o line reported by some of us, mice progressively developed ataxia and Purkinje cell loss. Here we report torpedo-like axonal swellings associated with residual Purkinje cells in Prnp o/o mice, and we demonstrate abnormal myelination in the spinal cord and peripheral nerves in mice from two independently established Prnp o/o lines. Mice were successfully rescued from both demyelination and Purkinje cell degeneration by introduction of a transgene encoding wild-type mouse cellular prion protein. These findings suggest that cellular prion protein expression may be necessary to maintain the integrity of the nervous system.
MH 3' Untranslated Regions; Alleles; Animal; Brain/*pathology; Demyelinating Diseases/*genetics/physiopathology/prevention & control; Exons; Hamsters; Introns; Mesocricetus; Mice; Mice, Mutant Strains; Mice, Transgenic; Motor Activity; Peripheral Nerves/pathology; Prions/*genetics/*physiology; Psychomotor Performance; Purkinje Cells/*pathology; Spinal Cord/pathology; Support, Non-U.S. Gov't; Vacuoles/pathology
AD Department of Bacteriology, Nagasaki University School of Medicine, Japan.
SP englisch
PO USA