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Neurodegeneration

Evolved protection against human prions

Nature volume 522, pages 423–424 (2015)Cite this article

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A genetic variant of PrP, the protein that forms prions, confers protection against the human prion disease kuru by inhibiting the conversion of functional isoforms to the abnormal, disease-causing conformation. See Letter p.478

The group of neurodegenerative disorders known as transmissible spongiform encephalopathies are incurable and lethal. They are caused by prions, a type of infectious agent that has challenged our understanding of the concepts of infection and inheritance. In prion diseases, an abnormal isoform of prion protein (PrP), dubbed PrPSc, induces conformational change in the functional isoform PrPC. The functional form adopts a disease-causing structure, and these abnormal proteins propagate throughout the brain, damaging nerves. But the mechanistic underpinnings of this infectious propagation remain enigmatic. On page 478 of this issue, Asante et al.1 describe a form of PrPC that seems to be resistant to such conformational change, providing some insight into the long-standing issue of whether it is possible to prevent prion diseases.

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Figure 1: Valine variants.

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  1. Glenn Telling is at the Prion Research Center, Colorado State University, Fort Collins, Colorado 80525, USA.,

    Glenn Telling

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  1. Glenn Telling
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Correspondence to Glenn Telling.

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Telling, G. Evolved protection against human prions. Nature 522, 423–424 (2015). https://doi.org/10.1038/nature14534

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