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Kuru, the First Human Prion Disease

2019/03/07 by Paweł P. Liberski, Agata Gajos, Beata Sikorska +1 · 1 citation
Biochemistry, Genetics and Molecular Biology · Neuroscience · #Prion Diseases and Protein Misfolding #Neurological diseases and metabolism

paper · pdf · doi:10.3390/v11030232

openalex publication_date 2019/03/07 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/22

Abstract

Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923⁻2008). In this review, we summarize the history of this seminal discovery, its anthropological background, epidemiology, clinical picture, neuropathology, and molecular genetics. We provide descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques retrieved from a paraffin-embedded block of an old kuru case, named Kupenota. The discovery of kuru opened new vistas of human medicine and was pivotal in the subsequent transmission of Creutzfeldt⁻Jakob 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 20th century.

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