Presentation 2002/3/13
Prion and Brain Damage
Kiyotoshi Kaneko,
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Abstract(in Japanese) (See Japanese page)
Abstract(in English) Prion protein exists in two different isoforms, a normal cellular isoform (PrP^C) and an abnormal infectious isoform (PrP^Sc), the latter is a causative agent of prion disease such as bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob (CJD) disease. Amino acid sequences of PrP^C and PrP^Sc are identical, but their conformations are rather different; PrP^C rich in non β-sheet vs. PrP^Sc rich in β-sheet isoform. Our prion research focuses on further understanding such an unprecedented mechanism by identifying auxiliary factor(s) other than PrP^C and PrP^Sc. Our current targets are, (1) yet unidentified protease cleaving PrP^C during its normal metabolic pathway, and (2) unknown host-specific factor(s) involved in PrP^Sc formation. These studies also help us to develop "therapeutics and prevention methods" in prion disease. Two trials including genetic manipulation with dominant negative mutant PrP^C gene working against the hypothetical host factor, or antibody therapy with anti-PrP antibodies which blocks PrP^C-PrP^Sc binding are currently underway.
Keyword(in Japanese) (See Japanese page)
Keyword(in English) Prion / Creutzfeldt-Jakob disease (CJD) / Bovine spongiform encephalopathy (BSE)
Paper # SSS2001-38
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Committee SSS
Conference Date 2002/3/13(1days)
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Language JPN
Title (in Japanese) (See Japanese page)
Sub Title (in Japanese) (See Japanese page)
Title (in English) Prion and Brain Damage
Sub Title (in English)
Keyword(1) Prion
Keyword(2) Creutzfeldt-Jakob disease (CJD)
Keyword(3) Bovine spongiform encephalopathy (BSE)
1st Author's Name Kiyotoshi Kaneko
1st Author's Affiliation Department of Cortical Function Disorders, National Institute of Neuroscience, National Center of Neurology and Psychiatry()
Date 2002/3/13
Paper # SSS2001-38
Volume (vol) vol.101
Number (no) 739
Page pp.pp.-
#Pages 6
Date of Issue