Promiscuous functions of the prion protein family

Promiscuous functions of the prion protein family

Sophie Mouillet-Richard* and Jean-Luc Vilotte 1 Toxicology, Pharmacology, and Cellular Signaling, Institut National de la Santé et de la Recherche Médicale Unité Mixtes de Recherche-S1124, Université Paris Descartes, Paris, France 2 Toxicology, Pharmacology, and Cellular Signaling, Sorbonne Paris Cité, Unité Mixtes de Recherche-S1124, Université Paris Descartes, Paris, France 3 Unité Mixtes de ...

Cytoprotective and toxic functions of the prion protein family

The prion protein family

Although the pivotal implication of the host-encoded Prion protein, PrP, in the neuropathology of transmissible spongiform encephalopathy is known for decades, its biological role remains mostly elusive. Genetic inactivation is one way to assess such issue but, so far, PrP-knockout mice did not help much. However, recent reports involving (1) further studies of these mice during embryogenesis, ...

Physiological Functions of the Cellular Prion Protein

The prion protein, PrPC, is a small, cell-surface glycoprotein notable primarily for its critical role in pathogenesis of the neurodegenerative disorders known as prion diseases. A hallmark of prion diseases is the conversion of PrPC into an abnormally folded isoform, which provides a template for further pathogenic conversion of PrPC, allowing disease to spread from cell to cell and, in some c...

Biochemical insight into the prion protein family

Prion protein family comprises proteins, which share not only similarity in their primary structure, but also similarity in their fold. These two groups of similarity presume a parceling in their respective biological function through the common biochemical properties. In this review, biochemical and structural similarities of PrP and two other proteins, Doppel and Shadoo, are evocated. Some ev...