NR AESI
AU Gomi,H.; Yokoyama,T.; Fujimoto,K.; Ikeda,T.; Katoh,A.; Itoh,T.; Itohara,S.
TI Mice devoid of the glial fibrillary acidic protein develop normally and are susceptible to scrapie prions
QU Neuron 1995 Jan; 14(1): 29-41
PT journal article
AB Glial fibrillary acidic protein (GFAP) is an intermediate filament protein specifically expressed in astrocytes in the CNS. To examine the function of GFAP in vivo, the Gfap gene was disrupted by gene targeting in embryonic stem cells. Mice homozygous for the mutation were completely devoid of GFAP but exhibited normal development and showed no obvious anatomical abnormalities in the CNS. When inoculated with infectious scrapie prions, the mutant mice exhibited neuropathological changes typical of prion diseases. Infectious prions accumulated in brains of the mutant mice to a degree similar to that in control littermates. These results suggest that GFAP is not essential for the morphogenesis of the CNS or for astrocytic responses against neuronal injury. The results argue against the hypothesis that GFAP plays a crucial role in the pathogenesis of prion diseases.
ZR 67
MH Animal; Astrocytes/pathology/physiology; Brain/growth & development; Brain Chemistry; Female; Gene Targeting; Glial Fibrillary Acidic Protein/genetics/*physiology; Male; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Mutagenesis; Prions/*metabolism; RNA, Messenger/analysis; Scrapie/*etiology/pathology; Spinal Cord/chemistry/growth & development; Support, Non-U.S. Gov't; Vimentin/metabolism; beta-Galactosidase/metabolism
AD Institute for Virus Research, Kyoto University, Japan.
SP englisch
PO USA