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Identification and structural analysis of C-terminally truncated collapsin response mediator protein-2 in a murine model of prion diseases
Authors:Fumiko Shinkai-Ouchi  Yoshio Yamakawa  Hideyuki Hara  Minoru Tobiume  Masahiro Nishijima  Kentaro Hanada  Ken'ichi Hagiwara
Institution:1.Department of Biochemistry and Cell Biology,National Institute of Infectious Diseases,Shinjuku-ku,Japan;2.Department of Pathology,National Institute of Infectious Diseases,Shinjuku-ku,Japan;3.National Institute of Health Sciences,Setagaya-ku,Japan
Abstract:

Background  

Prion diseases are fatal neurodegenerative disorders that accompany an accumulation of the disease-associated form(s) of prion protein (PrPSc) in the central nervous system. The neuropathological changes in the brain begin with focal deposits of PrPSc, followed by pathomorphological abnormalities of axon terminal degeneration, synaptic loss, atrophy of dendritic trees, and eventual neuronal cell death in the lesions. However, the underlying molecular basis for these neuropathogenic abnormalities is not fully understood.
Keywords:
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