Prion protein and the transmissible spongiform encephalopathies

doi:10.1016/S0962-8924(96)10054-4

Trends in Cell Biology

Volume 7, Issue 2, February 1997, Pages 56-62

https://doi.org/10.1016/S0962-8924(96)10054-4 Get rights and content

Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases that occur in a wide variety of mammals.

In humans, TSE diseases include kuru, sporadic and iatrogenic Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI). So far, TSE diseases occur only rarely in humans; however, scrapie is a widespread problem in sheep, and the recent epidemic of bovine spongiform encephalopathy (BSE or mad cow disease) has seriously affected the British cattle industry. Of special concern is the recent appearance of a new variant of CJD in humans that is suspected of being caused by infections from BSE-infected cattle products. In all these diseases, an abnormal form of a host protein, prion protein (PrP), is essential for the pathogenic process. The relationship of this protein to the transmissible agent is currently the subject of great interest and controversy and is the subject of this review.

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The cellular prion protein is a membrane bound glycoprotein expressed mainly in the Central Nervous System (CNS) of all mammals and avian species, although it is expressed throughout the body [2,6,7]. This protein is tethered to the outside surface of the cells through a glycosylphosphatidylinositol (GPI) anchor [8]. Generally, the normal cellular form is designated as PrPc and the pathogenic form responsible for the TSEs is designated as PrPSc.
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Trends in Cell Biology

ReviewPrion protein and the transmissible spongiform encephalopathies

Mol. Aspects Med.

Cell

J. Biol. Chem.

J. Biol. Chem.

Biochem. Biophys. Res. Commun.

J. Biol. Chem.

J. Biol. Chem.

J. Biol. Chem.

J. Biol. Chem.

J. Biol. Chem.

J. Biol. Chem.

Cell

Chem. Biol.

Eur. J. Pharmacol.

Neurodegeneration

Neurosci. Lett.

Brain Res.

Brain Res.

Neuroscience

Cell

Nature

Science

Acta Neuropathol.

Science

Nature

Nature

Arch. Virol.

J. Gen. Virol.

Methods in Molecular Medicine: Prion Diseases

Science

Br. Med. Bull.

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Prion protein and the transmissible spongiform encephalopathies