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AbstractChaperonins are molecular chaperones that play critical physiological roles, but they can be pathogenic. Malfunctional chaperonins cause chaperonopathies of great interest within various medical specialties. Although the clinical-genetic aspects of many chaperonopathies are known, the molecular mechanisms causing chaperonin failure and tissue lesions are poorly understood. Progress is necessary to improve treatment, and experimental models that mimic the human situation provide a promising solution. We present two models: one prokaryotic (the archaeon Pyrococcus furiosus) with eukaryotic-like chaperonins and one eukaryotic (Chaetomium thermophilum), both convenient for isolation-study of chaperonins, and report illustrative results pertaining to a pathogenic mutation of CCT5. Copyright 2019, The Author(s).
SponsorsF.T.R. is supported by Award 1819046 from the US National Science Foundation. M.Y. was supported by grants-in-aids for scientific research (JP16H04572, JP16H00753, and JP18H04690) from the Ministry of Education, Science, Sports, and Culture of Japan
and by Global Innovation Research Organization, Tokyo University of Agriculture and Technology
and MEXT/JSPS KAKENHI Grant numbers 16H04572 and 18H04690. A.J. L.M. and E.C.de M. were partially supported by IMET and IEMEST. This is IMET contribution number IMET 18-013.
Identifier to cite or link to this itemhttps://www.scopus.com/inward/record.uri?eid=2-s2.0-85071154536&doi=10.1038%2fs42003-019-0318-5&partnerID=40&md5=5be0c3dc1557ce18bff19326661f0d0b; http://hdl.handle.net/10713/10485