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OBSERVATIONS ON KURU : I. A POSSIBLE GENETIC BASIS
Author(s) -
BENNETT J. H.,
RHODES F. A.,
ROBSON H. N.
Publication year - 1958
Publication title -
australasian annals of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 0571-9283
DOI - 10.1111/imj.1958.7.4.269
Subject(s) - kuru , disease , genotype , incidence (geometry) , demography , biology , medicine , pathology , genetics , scrapie , gene , prion protein , physics , sociology , optics
Summary Kuru, a recently described neurological disease, occurs with high frequency among the Fore people, in a remote part of the Eastern Highlands of New Guinea. The results are given of a genealogical survey carried out in the northern Fore area. The survey recorded 2099 individuals, of whom 913 were dead. The cause of death was given as kuru in 204 instances, including 177 females (21 children) and 27 males (19 children). An analysis is presented which shows that male victims of kuru are born almost exclusively to mothers with the disease, and that there is a similar association between young female victims and their mothers. The incidence of the disease is almost equal in males and young females, while a larger proportion of adult females is affected. A tentative genetical hypothesis is advanced, suggesting that kuru may be controlled by a single autosomal gene “K”, which is recessive to its allelomorph “k” in males, and dominant in females. The females of genotype KK tend to have an early onset of the disease, while those of genotype Kk tend to have an onset in adult life.