So far there has been no cure for the fatal transmissible human prion diseases including the most common sporadic Creutzfeldt-Jakob disease (sCJD) that are characterized by the deposition of a misshaped infectious prion protein called (PrPSc) in the brain.
TC-5RW is a compound of cellulose ethers (CEs) that have already widely been used as inactive ingredients in foods and pharmaceuticals. Interestingly, our recent study has revealed for the first time that TC-5RW is able to directly decrease the amount of human PrPSc and inhibit PrPSc seeding activity in tissues from various sporadic and genetic CJD in vitro.
Therefore, it is most likely that CEs can be used for clinical trials of human prion diseases. For example, CEs may have a prophylactic effect on asymptomatic carriers of PrP mutations that are associated with various genetic prion diseases. Most of the asymptomatic PrP mutation carriers inevitably develop genetic prion diseases during aging.
In collaboration with Dr. Qingzhong Kong from Case Western Reserve University, Cleveland, Ohio, USA, and Dr. Ruth Gabizon, from Hadassah University Hospital, Jerusalem, Israel, this proposed study will test the efficacy of CE compound TC-5RW in the prevention and treatment of prion diseases with animal models that express human PrP infected with the most common subtype of sporadic CJD and an animal model of genetic CJD that develops prion disease spontaneously. This study is highly relevant to human prion diseases, especially to developing efficient prophylactic and therapeutic compounds for CJD.
About the Researchers:
Wenquan Zou, MD, PhD
Department of Pathology, Case Western Reserve University, Cleveland, Ohio
Dr. Wenquan Zou is a Professor from the Departments of Pathology and Neurology, and Associate Director of National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, Ohio.
Dr. Zou received his medical degree from Jiangxi Medical College and his Ph.D. from Shanghai Medical University, China. He has practiced Internal Medicine and Nephrology in China for years. His post-doctoral work in neurodegenerative diseases, with a concentration in prion diseases, was done at the Institute of Pathology, Case Western Reserve University, Cleveland, Ohio and at the Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Canada.
Dr. Zou’s research focus is in the area of prion disease and other neurodegenerative diseases such as Alzheimer’s disease and Parkinson’s disease. Currently, the Zou laboratory is developing prophylactic and therapeutic compounds for prion diseases and skin-based early diagnostic tests for prion disease and other neurodegenerative diseases.
Katsumi Doh-ura, MD PhD
Professor, Department of Neurochemistry, Tohoku University Graduate School of Medicine, Japan
Dr. Doh-ura studied neuropathology and genetics of human prion diseases when he was a graduate student of Jun Tateishi’s lab in Kyushu University, Japan, from 1987 to 1991. Thereafter, he worked as a postdoc at Bruce Chesebro’s lab in NIAID/NIH, USA, till 1994. He started the research on prion therapeutics when he obtained a position at Kyushu University in 1995. He joined Tohoku University as a faculty member in 2003, where he has continued his research on prion therapeutics.
- The Garry Buttermann, IV Memorial Research Grant, contributed by Jill Canuel
- The Dora Middendorf Memorial Grant contributed by Renee Middendorf and Family
- The Lynda Morris Memorial Grant, contributed by David Morris and Family
- The Eugene A. Riedel Memorial Research Grant, contributed by Jacqueline Riedel
- The Dr. Robert Brooks Memorial Grant, contributed by Loving Family & Friends
- The Paula Smart Memorial Research Grant, contributed by Jeff Moxie
- The Strides for CJD Grant, contributed by the Families of the CJD Foundation
- The CJD Foundation Grant, contributed by the Families of the CJD Foundation