Kuru human prion disease

Acquired human prion diseases include kuru and variant CJD 794 Prion protein polymorphism contributes genetic susceptibility to prion disease 794... 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

Acquired human prion diseases include kuru and variant... 

Fig. 3. Classification of human prion diseases. Sporadic the transformation from PrPc (circle) to PrPSc (square) occurs without apparent cause. Familial a point mutation ( ) is thought to facilitate the transformation. Infectious the transformation arises via PrPSc which acts as a template. The kinetic equations are defined by Eigen (1996). The infectious form includes kuru, iatrogenic CJD (iCJD), variant CJD (vCJD first reported in 1996), bovine spongiform encephalopathy (BSE first reported in 1985), and scrapie. In the nucleation-dependent model, monomeric PrPc and PrPSc are in chemical equilibrium.

The human prion diseases include CJD, kuru, Gerstmann-Straussler-Scheinker (GSS), and fatal insomnia, which can be classified into three groups based on etiology (Table 29.1). 

PrP " deposition in the central nervous system is also a hallmark feature of prion disease (Prusiner, 1991). The type of Prpsc cleposit and its location varies among the human prion diseases. PrP is also a component of amyloid plaques. A characteristic of vCJD and kuru is florid amyloid plaques, which is a large cluster of PrP aggregates interspersed with vacuolar pathology. PrP deposition is also present in peripheral tissues of patients with vCJD, primarily in secondary... 

The acquired prion diseases account for less than 1 % of all human prion disease cases and these include variant CJD, iatrogenic CJD (iCJD), and kuru (reviewed by Will, 2003). In these cases, prion infection is either orally acquired or associated with accidental transmission via medical practices. Many of these latter cases involve contamination with brain tissue, which contains the highest amount of prion infectivity, from the donor host. 

Further evidence supporting Hectd2 as a candidate gene was provided in an association study of HECTD2 SNPs in human prion diseases [68]. From the UK, patients with variant CJD and sporadic CJD were genotyped and compared to a control population. From PNG, kuru patients were genotyped and compared to... 

Kuru, a human prion disease formerly transmitted by ritual cannibalism in New Guinea, is histologically indistinguishable from sCJD in individual cases however, it has been noted that kuru shows considerable similarities to sCJD VV2 or sCJD MV 2K [29, 94-97],... 

Collinge J, Whitfield J, McKintosh E, Beck J, Mead S, Thomas DJ, Alpers MP (2006) Kuru in the 21st century-an acquired human prion disease with very long incubation periods. Lancet 367 2068-2074... 

Known human prion diseases are Creutzfeldt-Jacob disease (CJD), Gerstmann-Straussler-Schienker syndrome (GSS) and fatal familial insomnia (FFI). GSS and FFI are inherited, whereas CJD may be inherited, sporadic or infectious. Another infectious form of CJD (iatrogenic CJD) arises from inadequately sterilized surgical instruments, dura mater grafts, and from human growth hormone isolated from cadavers. Kuru is a classical example of an infectious human prion disease transmitted by the ritual cannibalism of human brains, this disease was formerly common in the Fore tribe of New Guinea. 

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