Kuru: A Journey Back in Time from Papua New Guinea to the Neanderthals’ Extinction

Abstract

Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923. In this review, I briefly summarize the history of this seminal discovery along 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-Strä ussler-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 20th century.

Routes of Transmission in the Food Chain

“…As of 2011, all but one individual who developed vCJD were homozygous at codon 129 for methionine, and a single heterozygous individual was reported (Mead et al, 2009;Colby and Prusiner, 2011). Another transmissible spongiform encephalopathy, kuru, a neurodegenerative disease described in Papua New Guinea, had an incubation period sometimes exceeding 50 years and was transmitted by ritualistic endocannibalism (which refers to the eating of relatives, as opposed to the eating of enemies, known as exocannibalism) (Gajdusek, 1977;Colby and Prusiner, 2011;Liberski, 2013). Kuru was restricted to members of the Foré linguistic group and neighboring groups but did not affect groups into which the kuru-affected people did not intermarry.…”

“…Kuru was restricted to members of the Foré linguistic group and neighboring groups but did not affect groups into which the kuru-affected people did not intermarry. In the Foré language, the word "kuru" means "to tremble with fear or cold" (Liberski, 2013). The incidence of kuru started to decline after the 1950s when endocannibalism had gradually stopped (Collinge et al, 2006).…”

“…The incidence of kuru started to decline after the 1950s when endocannibalism had gradually stopped (Collinge et al, 2006). Studies that examined the molecular genetic basis of susceptibility to kuru found that homozygotes for methionine at codon 129 were overrepresented in the younger age group, while valine homozygotes and heterozygotes were overrepresented in the much older age group, and survivors were almost never carried homozygotes for methionine at this position (Liberski, 2013). Heterozygosity at this codon was associated with older patients and with longer incubation times (Mead et al, 2008).…”

Manganese and Prion Disease

“…40 An evolutionarily strong balancing selection for these alleles had been imposed at this locus, not only in Fore, but also in other human populations practicing cannibalism. 41 autonomic hyperactivation, and cognitive and motor impairments such as dysarthria, myoclonus, ataxia, tremor, and pyramidal and extrapyramidal signs. 54 Thalamic atrophy is recognized as the histopathological hallmark of FFI, while pathological lesions in the neocortex and the limbic cortex are also observed.…”

Amylotrophic Lateral Sclerosis-Like Motor Impairment in Prion Diseases

“…Apart from those, Prion diseases are characterized as the lethal form of neurodegenerative diseases with no clearly defined molecular mechanism and cure. Among the different types of Prion diseases, Kuru is one of the oldest that was discovered in New Guinea [2]. Later, Creudzfelt-Jacob Disease (CJD) was identified for the first time in the UK in late 1990s [3].…”