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Kuru: Introduction to Prion Diseases

  • Paweł P. LiberskiEmail author
  • Agata Gajos
Protocol
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Part of the Neuromethods book series (NM, volume 129)

Abstract

Kuru, the first human transmissible spongiform encephalopathy, was transmitted to chimpanzees in the D. Carleton Gajdusek (1923–2008) laboratory. In this review, we briefly summarize the history of this seminal discovery along with its epidemiology, clinical picture, neuropathology, and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine, and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease, as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the twentieth century.

Key words

Kuru Prion diseases Neuropathology D. Carleton Gajdusek 

Notes

Acknowledgements

I am immensely indebted to late Dr. D. Carleton Gajdusek and Dr. Clarence J. Gibbs Jr. for generously providing me unique illustrations I have used through the text. I thank Prof. Shirley Lindenbaum and Prof. Michael Alpers for exciting discussion and helpful criticism, and Prof. James W. Ironside, the National CJD Surveillance Unit, for reading and correcting the MS in totally impossibly express tempo.

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© Springer Science+Business Media LLC 2017

Authors and Affiliations

  1. 1.Laboratory of Electron Microscopy and Neuropathology, Department of Molecular Pathology and NeuropathologyMedical University LodzLodzPoland
  2. 2.Department of Extrapyramidal DiseasesMedical University of LodzLodzPoland

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