Identification and structural analysis of C-terminally truncated collapsin response mediator protein-2 in a murine model of prion diseases |
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Authors: | Fumiko Shinkai-Ouchi Yoshio Yamakawa Hideyuki Hara Minoru Tobiume Masahiro Nishijima Kentaro Hanada Ken'ichi Hagiwara |
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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 |
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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. |
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