The function of the cellular prion protein in health and disease
The essential role of the cellular prion protein (PrP C) in prion disorders such as Creutzfeldt–
Jakob disease is well documented. Moreover, evidence is accumulating that PrP C may act …
Jakob disease is well documented. Moreover, evidence is accumulating that PrP C may act …
The PrP-like proteins Shadoo and Doppel
An almost unique place within protein databases, twenty-five years of study has
underscored the enigmatically subtle role of PrP C in normal cell biology. It seems that PrP …
underscored the enigmatically subtle role of PrP C in normal cell biology. It seems that PrP …
The prion protein is critical for DNA repair and cell survival after genotoxic stress
A Bravard, F Auvré, D Fantini… - Nucleic acids …, 2015 - academic.oup.com
The prion protein (PrP) is highly conserved and ubiquitously expressed, suggesting that it
plays an important physiological function. However, despite decades of investigation, this …
plays an important physiological function. However, despite decades of investigation, this …
Structural features within the nascent chain regulate alternative targeting of secretory proteins to mitochondria
NV Pfeiffer, D Dirndorfer, S Lang… - The EMBO …, 2013 - embopress.org
Protein targeting to specified cellular compartments is essential to maintain cell function and
homeostasis. In eukaryotic cells, two major pathways rely on N‐terminal signal peptides to …
homeostasis. In eukaryotic cells, two major pathways rely on N‐terminal signal peptides to …
Exacerbation of experimental autoimmune encephalomyelitis in prion protein (PrPc)-null mice: evidence for a critical role of the central nervous system
P Gourdain, C Ballerini, AB Nicot… - Journal of …, 2012 - Springer
Background The cellular prion protein (PrPc) is a host-encoded glycoprotein whose
transconformation into PrP scrapie (PrPSc) initiates prion diseases. The role of PrPc in …
transconformation into PrP scrapie (PrPSc) initiates prion diseases. The role of PrPc in …
Protease-resistant prions selectively decrease Shadoo protein
The central event in prion diseases is the conformational conversion of the cellular prion
protein (PrPC) into PrPSc, a partially protease-resistant and infectious conformer. However …
protein (PrPC) into PrPSc, a partially protease-resistant and infectious conformer. However …
Neuroprotective and neurotoxic signaling by the prion protein
UK Resenberger, KF Winklhofer, J Tatzelt - Prion Proteins, 2011 - Springer
Prion diseases in humans and animals are characterized by progressive neurodegeneration
and the formation of infectious particles called prions. Both features are intimately linked to a …
and the formation of infectious particles called prions. Both features are intimately linked to a …
Down-Regulation of Shadoo in Prion Infections Traces a Pre-Clinical Event Inversely Related to PrPSc Accumulation
D Westaway, S Genovesi, N Daude, R Brown… - PLoS …, 2011 - journals.plos.org
During prion infections of the central nervous system (CNS) the cellular prion protein, PrPC,
is templated to a conformationally distinct form, PrPSc. Recent studies have demonstrated …
is templated to a conformationally distinct form, PrPSc. Recent studies have demonstrated …
Endoproteolytic processing of the mammalian prion glycoprotein family
Cellular prion protein (P r PC) misfolds to form infectivity‐associated scrapie prion protein
and generates C‐terminal fragments C 1 and C 2 in healthy and prion‐infected animals. C1 …
and generates C‐terminal fragments C 1 and C 2 in healthy and prion‐infected animals. C1 …
Dimerization of the cellular prion protein inhibits propagation of scrapie prions
AD Engelke, A Gonsberg, S Thapa, S Jung… - Journal of Biological …, 2018 - ASBMB
A central step in the pathogenesis of prion diseases is the conformational transition of the
cellular prion protein (PrP C) into the scrapie isoform, denoted PrP Sc. Studies in transgenic …
cellular prion protein (PrP C) into the scrapie isoform, denoted PrP Sc. Studies in transgenic …