The prion protein family: Diversity, rivalry, and dysfunction

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, ...

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 ...

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...

Cytoprotective and toxic functions of the prion protein family

The prion protein family: a view from the placenta

Based on its developmental pattern of expression, early studies suggested the implication of the mammalian Prion protein PrP, a glycosylphosphatidylinositol-anchored ubiquitously expressed and evolutionary conserved glycoprotein encoded by the Prnp gene, in early embryogenesis. However, gene invalidation in several species did not result in obvious developmental abnormalities and it was only re...