Department of
Medical Microbiology
Representative:
Professor Noriyuki Nishida
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We have studied on the transmissible spongiform encephalopathy caused by a mysterious infectious agent, so-called prion.
A variety of the diseases has been known in mammals, Creutzfeldt-Jakob Disease (CJD) in humans, Bovine Spongiform Encephalopathy (BSE) in cattle, Scrapie in sheep/gout, or Chronic Wasting Disease (CWD) in wild deer.
Although the outbreak of BSE, which started in 80's of UK and spread world wide, has now been settled, CWD are spreading among wild life of the world.
For this 30 years, it has been elucidated that the diseases are caused by abnormally folded prion protein (PrP) and the accumulation of the PrP-amyloid is pathological feature of the diseases and the proteins are infectious, but CJD in humans is still untreatable and fatal. We are particularly interested in (1) protective role of innate immunity during prion infection, (2) underlying mechanisms for the prion diversity and PrP-conversion process. (3) degaradation pathway of the proteins. We also conducting the drug discovery for human prion diseasesa using supercomputer DEGIMA. We have successfully developed a new diagnostic test of the diseases, that can achieve accurate early diagnosis using in vitro amplification method of prion. This RT-QuIC assay is powerful and useful, and we are now able to detect very small amount of prions in the cerebrospinal fluid and also in non-neural tissues from the patients. Our goal is to understand the mysterious prion and to establish early diagnosis and effective treatments for the fatal diseases. |
