Kuru disease

Kuru disease is one of a family of diseases known as prion diseases because they are caused by an abnormal form of the prion (PrP) protein found in membranes (Shetty and Steele 1). The family includes Creutzfeldt-Jacob disease (CJD), Gerstmann-Straussler-Schneinker syndrome (GSS), and fatal familial insomnia, as well as scrapie and bovine spongiform encephalopathy (BSE - mad cow disease) (Brown, Goldfarb and Gajdusek 1019; Prusiner 264; Shetty and Steel 1). A unique characteristic common to all these diseases is that they involve aberrant metabolism of the prion protein, and in many cases the cellular prion protein is converted into the scrapie variant by a process of conformational change. The prion disease all segregate with prion protein gene mutations.

Three hypotheses have been advanced to explain human spongiform encephalopathies: 1)an exogenous nucleic acid-containing pathogen, with the host amyloid protein as a sequela of infection; 2)an exogenous nucleic acid wrapped in the host protein; and 3) the protein can self-replicate (Brown, Goldfarb and Gajdusek 1020).

Human prion disease occur as infectious (Kuru, CJD), sporadic, and familial disorders depending on the clinical and neuropathologic findings (Prusiner 265). CJD has its onset in midlife, causing dementia, loss of muscular coordination, and other neurological abnormalities, and is usually fatal within one year (Brown, Goldfarb and Gajdusek 1019). GSS is a variant of

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m of prion protein) is converted to PrPsc (scrapie isoform of prion protein) by templated infolding. The prototype prion protein (PrP) was discovered in studies of experimental scrapie disease in rodents (Mastrangelo and Westaway 613). Closely related molecules underlie the recent epidemic of bovine encephalopathy in Britain, and other prion proteins have been associated with inheritable cytoplasmic traits in yeasts and fungi. Unlike the classical vectors of infectious disease in humans - bacteria and viruses - mammalian prion proteins are encoded by the host genes, and not by an extrinsic nucleic acid genome. Prion proteins come in two forms. PsPc (cellular PrP) is benign and is probably present in all mammals, where it is expressed on the surface of neurons via a GPI anchor. Nuclear magnetic resonance (NMR) studies have revealed that recombinant mouse, hamster, bovine, and human prion proteins that approximate PrPC have a globular C-terminal domain with three ?-helices and two short [-strands, preceded by a larger N-terminal region that has not yet been structured. The protease-resistant, infectious form of the prion protein, PrPs, has a different structure rich in the [-sheet folding motif. PrPc is labile, both in v

Category: Medical - K