The biological function of the cellular prion protein: an update

MA Wulf, A Senatore, A Aguzzi - BMC biology, 2017 - Springer
The misfolding of the cellular prion protein (PrP C) causes fatal neurodegenerative
diseases. Yet PrP C is highly conserved in mammals, suggesting that it exerts beneficial …

Physiology of the prion protein

R Linden, VR Martins, MAM Prado… - Physiological …, 2008 - journals.physiology.org
Prion diseases are transmissible spongiform encephalopathies (TSEs), attributed to
conformational conversion of the cellular prion protein (PrPC) into an abnormal conformer …

Prion protein is necessary for normal synaptic function

J Collinge, MA Whittington, KCL Sidle, CJ Smith… - Nature, 1994 - nature.com
THE prion diseases are neurodegenerative conditions, transmissible by inoculation, and in
some cases inherited as an autosomal dominant disorder. They include Creutzfeldt–Jakob …

Regulation of affect by the lateral septum: implications for neuropsychiatry

TP Sheehan, RA Chambers, DS Russell - Brain Research Reviews, 2004 - Elsevier
Substantial evidence indicates that the lateral septum (LS) plays a critical role in regulating
processes related to mood and motivation. This review presents findings from the basic …

Copper stimulates endocytosis of the prion protein

PC Pauly, DA Harris - Journal of Biological Chemistry, 1998 - ASBMB
Prion diseases result from conformational alteration of PrP C, a cell surface glycoprotein
expressed in brain, spinal cord, and several peripheral tissues, into PrP Sc, a protease …

Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions

D Shmerling, I Hegyi, M Fischer, T Blättler, S Brandner… - Cell, 1998 - cell.com
The physiological role of prion protein (PrP) remains unknown. Mice devoid of PrP develop
normally but are resistant to scrapie; introduction of a PrP transgene restores susceptibility to …

Early accumulation of PrPSc in gut-associated lymphoid and nervous tissues of susceptible sheep from a Romanov flock with natural scrapie

O Andréoletti, P Berthon, D Marc… - Journal of General …, 2000 - microbiologyresearch.org
The immune system is known to be involved in the early phase of scrapie pathogenesis.
However, the infection route of naturally occurring scrapie and its spread within the host are …

Developmental expression of the prion protein gene in glial cells

M Moser, RJ Colello, U Pott, B Oesch - Neuron, 1995 - cell.com
Replication of prions is dependent on the presence of the host protein PrP c. During the
course of disease, PrP c is converted into an abnormal isoform, PrP s¢, which accumulates …

Evidence of presynaptic location and function of the prion protein

J Herms, T Tings, S Gall, A Madlung… - Journal of …, 1999 - Soc Neuroscience
The prion protein (PrPC) is a copper-binding protein of unknown function that plays an
important role in the etiology of transmissible spongiform encephalopathies. Using …

A vector for expressing foreign genes in the brains and hearts of transgenic mice

DR Borchelt, J Davis, M Fischer, MK Lee… - Genetic analysis …, 1996 - Elsevier
An expression plasmid (MoPrP. Xho), for use in transgenic mice, was developed from the
promoter, 5′ intronic, and 3′ untranslated sequences of the murine prion protein gene …