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Spongiform encephalopathies

Breech-birth prions

Nature volume 402, pages 737–739 (1999)Cite this article

Creutzfeldt–Jakob disease, Gerstmann–Straussler–Scheinker (GSS) syndrome, scrapie and bovine spongiform encephalopathy are part of a family of neurodegenerative diseases caused by changes in the metabolism of a plasma-membrane protein, PrPC or prion protein. These prion diseases can be acquired by infection, arise sporadically by an unknown mechanism, or result from inheritance of a defective PrP gene. An abnormal PrP (PrPSc) is characteristic of most prion diseases, but in some inherited cases the PrP that accumulates in the brain is not PrPSc but CtmPrP, a transmembrane form of the protein1. And on page 822 of this issue, Hegde and co-workers2 show that CtmPrP may be a neurotoxic molecule that is common to both the genetic and the acquired forms of prion diseases.

The prion hypothesis holds that an abnormal prion protein (PrPSc) may be the sole component of the ‘transmissible particle’ that causes acquired forms of disease. But there are several anomalies. For example, high levels of a GSS-syndrome mutant PrP (P101L) in the brains of transgenic mice (the Tg[MoPrP(P101L)] strain) induced a spontaneous neurological disorder in the absence of conventional PrPSc, although this disorder could be transmitted to a limited range of host rodents3. A subset of people with GSS syndrome has been found with a different mutation (A117V) in the PrP gene that also does not produce conventional PrPSc. However, this form of the disease cannot be transmitted to laboratory rodents4. Further anomalies in the prion hypothesis have been exposed by cross-species transmission experiments5,6, in which recipient mice develop disease without the formation of recognizable PrPSc.

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Figure 1: Possible role of CtmPrP in cell death.

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  1. the Institute for Animal Health, Compton Laboratory, Compton, Newbury, Berkshire, RG20 7NN, UK

    James Hope

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Correspondence to James Hope.

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Hope, J. Breech-birth prions. Nature 402, 737–739 (1999). https://doi.org/10.1038/45413

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