Prion-related protein

This volume of Advances in Protein Chemistry provides detailed and authoritative reviews of the current approaches to understanding the various prion-related proteins and the challenges that remain in... 

J. Pammer, W. Weninger and E. Tschachler, Human keratinocytes express cellular prion-related protein in vitro and during inflammatory skin diseases, Am. J. Pathol., 1998, 153(5), 1353-8. 

Kryndushkin D, Shewmaker F, Wickner R (2008) Curing of the URP3J prion by Btn2p, a Batten disease-related protein. EMBO J 27 2725-2735... 

The observations summarized in Section III.A and III.B suggests that the filaments detected in vivo are, in fact, the prion and that the filaments assembled in vitro from purified protein are the same as or closely related to the in vivo material. To substantiate this proposition, it is important to show that filaments formed in vitro are, in fact, infectious. Such infectivity has now been demonstrated for HET-s (Maddelein et al, 2002), Sup35p (King and Diaz-Avalos, 2004 Tanaka et al., 2004), and Ure2p (Fig. 6 Brachmann etal., 2005). In vitro -assembled filaments were brought into cells by biolistic... 

Recently, a seven-residue peptide from the Sup35p prion domain has been analyzed by X-ray crystallography (Nelson et al., 2005). Although there is, in general, little reason to suppose that a short peptide will assume the same structure in a crystal as it will in the context of a folded protein containing it, these crystals seem to be related to amyloid fibrils of the same... 

With the background of the mad cow crisis in Europe, questions relating to the prion diseases have attracted intensive interest. It is now widely accepted that prion diseases, such as Creutzfeldt-Jakob disease (CJd) in humans and bovine spongiform encephalopathy (BSE) are caused by a conformational change of the prion protein (PrP) from a normally folded cellular form, PrP ", to an alternate, aggregation-prone, pathogenic scrapie form,... 

Recently, high-pressure NMR measurements have enabled us to identify an intermediate conformer of the hamster prion protein, which has been suggested to be the PrP factor responsible for the PrP /PrP ° conversion or a closely related precursor. ... 

Observation of living systems shows them to be complex mixtures of different chemicals each of which functions to support one or more of the necessary tasks required to keep the organism alive . At one end of the spectrum of complexity lie the viruses and related simple proteins, such as prions, which cannot replicate by themselves but reproduce with the aid of other, higher, life forms. They therefore fulfill the definition of life to a very limited extent. At the other end of the spectrum there are the multicellular organisms such as humans with a vast diversity of processes that relate directly, and often more subtly, to the basic requirements of life. Even here, though, there is often a dependence on another living system. For example, humans cannot survive without vitamin Bi2 but are unable to synthesize it and must rely on external agents for its production. 

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