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First published online July 23, 2007

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Prion protection

Many neurodegenerative diseases are caused by the accumulation of insoluble protein aggregates. Transmissible spongiform encephalopathies (such as scrapie, BSE and Creutzfeldt-Jacob disease) occur when nerve cells are exposed to misfolded prion protein (PrP^Sc), which converts normal prion protein (PrP^C) into aggregates of PrP^Sc. But what causes the loss of neurons – is it just the presence of PrP^Sc or also the lack of PrP^C? Evan Eisenberg and colleagues (p. 2663) have been investigating this question in cultured human neuronal cells. The researchers manipulated PrP^C levels in these cells by knocking PrP^C down by RNAi or by overexpressing it by transfecting cells with a mouse prion gene. Then they examined the effects on protein aggregation by transfecting the cells with a version of the huntingtin protein that has an expanded polyglutamine tract and so is prone to aggregation. Cells lacking PrP^C display increased huntingtin aggregation; in cells overexpressing PrP^C, aggregation is reduced. Furthermore, the cells lacking PrP^C have reduced proteosome activity, reduced activity of cellular defence enzymes and more reactive oxygen species, leading the researchers to suggest that PrP^C protects cells from the toxic effects of aggregated huntingtin.

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Related articles in JCS:

Cellular prion protein (PrP^C) protects neuronal cells from the effect of huntingtin aggregation

Kyung-Jin Lee, Antony Panzera, David Rogawski, Lois E. Greene, and Evan Eisenberg
JCS 2007 120: 2663-2671. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in JCS Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?