Results 21 to 30 of about 33,090 (271)

Analysis of the prion protein gene in thalamic dementia [PDF]

Neurology, 1992
Thalamic degenerations or dementias are poorly understood conditions. The familial forms are (1) selective thalamic degenerations and (2) thalamic degenerations associated with multiple system atrophy. Selective thalamic degenerations share clinical and pathologic features with fatal familial insomnia, an autosomal dominant disease linked to a mutation
R. B. Petersen, M. Tabaton MD, L. Berg MD, B. Schrank MD, R. M. Torack, S. Leal MS, J. Julien MD, C. Vital MD, B. Deleplanque MD, W. W. Pendlebury, D. Drachman MD, T. W. Smith, J. J. Martin, M. Oda MD, P. Montagna MD, J. Ott PhD, L. Autilio-Gambetti PhD, E. Lugaresi MD, P. Gambetti MD   +18 more
openaire   +3 more sources

Prion pathogenesis is unaltered in the absence of SIRPα-mediated "don't-eat-me" signaling. [PDF]

PLoS ONE, 2017
Prion diseases are neurodegenerative conditions caused by misfolding of the prion protein, leading to conspicuous neuronal loss and intense microgliosis.
Mario Nuvolone, Marta Paolucci, Silvia Sorce, Veronika Kana, Rita Moos, Takashi Matozaki, Adriano Aguzzi   +6 more
doaj   +1 more source

ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver

Hepatology, EarlyView., 2022
Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid, Victor Olsavszky, Manuel Reinhart, Vanessa Weyer, Felix A. Trogisch, Carsten Sticht, Manuel Winkler, Sina W. Kürschner, Johannes Hoffmann, Roxana Ola, Theresa Staniczek, Joerg Heineke, Beate K. Straub, Jens Mittler, Kai Schledzewski, Peter ten Dijke, Karsten Richter, Steven Dooley, Cyrill Géraud, Sergij Goerdt, Philipp‐Sebastian Koch   +20 more
wiley   +1 more source

Prion protein self-peptides modulate prion interactions and conversion [PDF]

, 2009
Background: Molecular mechanisms underlying prion agent replication, converting host-encoded cellular prion protein (PrPC) into the scrapie associated isoform (PrPSc), are poorly understood.
Bossers, A., Fred G van Zijderveld, Priem, J., Drophatie Timmers-Parohi, Rigter, A., Alex Bossers, Alan Rigter, Timmers-Parohi, D., Jan PM Langeveld, van Zijderveld, F.G., Zijderveld, F.G., van, Jan Priem, Langeveld, J.P.M.   +12 more
core   +1 more source

Promiscuous Functions of the Prion Protein Gene Family [PDF]

, 2015
The cellular prion protein PrPC is a ubiquitous GPI-anchored protein. While PrPC has been the focus of intense research for its involvement in a group of neurodegenerative disorders known as transmissible spongiform encephalopathies (TSE), much less attention has been devoted to its physiological function.
Mouillet-Richard, Sophie, Vilotte, Jean-Luc   +1 more
openaire   +4 more sources

Genetic variability of the prion protein gene (PRNP) in wild ruminants from Italy and Scotland [PDF]

, 2009
The genetics of the prion protein gene (PRNP) play a crucial role in determining the relative susceptibility to transmissible spongiform encephalopathies (TSEs) in several mammalian species.
Acutis, Pier Luigi, Pier Luigi Acutis, Ezio Ferroglio, Simone Peletto, Hugh W. Reid, Perucchini, Matteo, Serena Robetto, Acin, Cristina, Bozzetta, Elena, Reid, Hugh W., Dalgleish, Mark P., Orusa, Riccardo, Elena Bozzetta, Ferroglio, Ezio, Sacchi, Paola, Paula Stewart, Daniela Meloni, Wilfred Goldmann, Meloni, Daniela, Gennero, Silvia, Riccardo Orusa, Roberto Rasero, Rasero, Roberto, Cristina Acín, Matteo Perucchini, Mark P. Dalgleish, Goldmann, Wilfred, Stewart, Paula, Maria Caramelli, Silvia Gennero, Caramelli, Maria, Robetto, Serena, Peletto, Simone, Paola Sacchi   +33 more
core   +1 more source

Is prnt a pseudogene? Identification of ram Prt in testis and ejaculated spermatozoa.

PLoS ONE, 2012
A hallmark of prion diseases or transmissible spongiform encephalopaties is the conversion of the cellular prion protein (PrP(C)), expressed by the prion gene (prnp), into an abnormally folded isoform (PrP(Sc)) with amyloid-like features that causes ...
Jorge Pimenta, Ana Domingos, Pedro Santos, Carla C Marques, Cátia Cantante, Ana Santos, João P Barbas, Maria C Baptista, António E M Horta, Aldino Viegas, Patrícia Mesquita, João Gonçalves, Carlos A Fontes, José A M Prates, Rosa M L N Pereira, Rosa M L N Pereira   +15 more
doaj   +1 more source

Gene expression profiling en association with prion-related lesions in the medulla oblongata of symptomatic natural scrapie animals. [PDF]

, 2011
The pathogenesis of natural scrapie and other prion diseases remains unclear. Examining transcriptome variations in infected versus control animals may highlight new genes potentially involved in some of the molecular mechanisms of prion-induced ...
Bossers, A., Rosa Bolea, Hicham Filali, Varona, L., Filalil, Hicham, Harders, F., Inmaculada Martin-Burriel, Alex Bossers, Zaragoza, P., Badiola, J.J., Juan J. Badiola, Bossers, Alex, Pumarola i Batlle, Martí, Lyahyai, Jaber, Lyahyai, J., Martín Burriel, Inmaculada, Pilar Zaragoza, Juan J Badiola, Bolea, Rosa, Filali, H., Bolea, R., Acín, Cristina, Luis Varona, Frank Harders, Filali, Hicham, Pumarola, M., Harders, Frank, Zaragoza, Pilar, Badiola, Juan J, Varona, Luis, Martí Pumarola, Jaber Lyahyai, Martin-Burriel, I., Badiola, Juan José, Pumarola, Martí   +34 more
core   +1 more source

Gene-Edited Cell Models to Study Chronic Wasting Disease

Viruses, 2022
Prion diseases are fatal infectious neurodegenerative disorders affecting both humans and animals. They are caused by the misfolded isoform of the cellular prion protein (PrPC), PrPSc, and currently no options exist to prevent or cure prion diseases ...
Simrika Thapa, Cristobal Marrero Winkens, Waqas Tahir, Maria I. Arifin, Sabine Gilch, Hermann M. Schatzl   +5 more
doaj   +1 more source

Proteinase K-Resistant Material in ARR/VRQ Sheep Brain Affected with Classical Scrapie Is Composed Mainly of VRQ Prion Protein [PDF]

, 2011
Classical scrapie is a prion disease in sheep and goats. In sheep, susceptibility to disease is genetically influenced by single amino acid substitutions.
Bossers, A., A. Bossers, T. Sklaviadis, I. Lantier, F. Lantier, L. J. M. van Keulen, Van Keulen, L., S. McCutcheon, Berthon, P., Lantier, Isabelle, van Keulen, L.J.M., van Zijderveld, F.G., P. Berthon, Lantier, Frédéric, F., Zijderveld, F.G., van, Jacobs, J.G., Keulen, L., Van, Langeveld, J.P.M., Lantier, I., H. Rezaei, J. P. M. Langeveld, Rezaei, Human, Sklaviadis, T., Berthon, Patricia, J. G. Jacobs, F. G. van Zijderveld, Lantier, F., McCutcheon, S., Rezaei, H.   +28 more
core   +1 more source