Results 41 to 50 of about 4,898,785 (204)

Analysis of the hippocampal proteome in ME7 prion disease reveals a predominant astrocytic signature and highlights the brain-restricted production of clusterin in chronic neurodegeneration

, 2014
Prion diseases are characterized by accumulation of misfolded protein, gliosis, synaptic dysfunction, and ultimately neuronal loss. This sequence, mirroring key features of Alzheimer disease, is modeled well in ME7 prion disease.
Asuni, Ayodeji A, Skipp, Paul, Gray, Bryony, Bailey, Joanne, O'Connor, Vincent, Perry, V Hugh   +5 more
core   +1 more source

Beyond PrP res type 1/type 2 dichotomy in Creutzfeldt-Jakob disease [PDF]

, 2008
Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres)identified on Western blotting ...
Ironside, James W, Uro-Coste, Emmanuelle, Grassi, J., Head, Mark, Jean-Marc Bilheude, Lacroux, Caroline, Cassard Hervé, Armand Perret-Liaudet, Marie Bernadette Delisle, Grassi, Jacques, Head, M.W., Hauw, J.J., Head Mark W., Andréoletti Olivier, Bilheude, Jean-Marc, Peoch, Katell, Delisle, Marie-Bernadette, Streichenberger, Nathalie, Ironside, J.E., Simon Stéphanie, Lacroux Caroline, Simon, S., Haik, S., Peoch' Katell, Hervé Cassard, Jean-Jacques Hauw, Haik Stéphane, Basset-Leobon Christelle, Bilheude, J.M., Andreoletti, O., Jan Langeveld, Bilheude Jean-Marc, Stéphanie Simon, Uro-Coste Emmanuelle, Schelcher, F., Ironside, James, W., Langeveld, Jan, Christelle Basset-Leobon, Jacques Grassi, Olivier Andréoletti, Lugan, Séverine, Andréoletti, Olivier, Emmanuelle Uro-Coste, Lugan, S., Caroline Lacroux, Ironside, James, Hauw, Jean-Jacques, Perret-Liaudet, Armand, Basset-Leobon, Christelle, Basset-Leobon, C., Cassard, Hervé, Stéphane Haik, Head, Mark, W., Hauw Jean-Jacques, Langeveld Jan, James W Ironside, Simon, Stéphanie, Peoch, K., Séverine Lugan, Streichenberger Nathalie, Langeveld, J.P.M., Perret-Liaudet, A., Streichenberger, N., Cassard, H., Delisle, M.B., Uro-Coste, E., Haik, Stéphane, Schelcher, Francois, Grassi Jacques, Peoc’h, Katell, Mark W Head, Delisle, Marie Bernadette, Lacroux, C., Lugan Séverine, Schelcher Francois, Delisle Marie Bernadette, Head, Mark W, Ironside James W., Nathalie Streichenberger, Francois Schelcher, Katell Peoch', Perret-Liaudet Armand, Peoch', Katell, Head, Mark,, Ironside, James,   +84 more
core   +1 more source

Sheep Are Susceptible to the Bovine Adapted Transmissible Mink Encephalopathy Agent by Intracranial Inoculation and Have Evidence of Infectivity in Lymphoid Tissues

Frontiers in Veterinary Science, 2019
Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agents of TME and L-BSE.
Eric D. Cassmann, S. Jo Moore, Jodi D. Smith, Justin J. Greenlee   +3 more
doaj   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Vassallo, Neville, Herms, Jochen, Kretzschmar, Hans A.   +2 more
core  

Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]

, 2013
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
Jeremy C. Simpson (29225), Franke, Markus, Sibeal Waldron (277507), Appelhans Dietmar, Rogers Mark S., Dietmar Appelhans (277509), Resenberger, Ulrike K., Simpson Jeremy C., Simpson, Jeremy C., Jörg Tatzelt, Franke Markus, Resenberger Ulrike K., Rogers, Mark S., McCarthy James M., Mark S. Rogers (277510), James M McCarthy, Sibeal Waldron, Dietmar Appelhans, Appelhans, Dietmar, Mark S Rogers, Tatzelt Jörg, Tatzelt, Jörg, Ulrike K. Resenberger (277506), McCarthy, James M., Waldron, Sibeal, Markus Franke, Ulrike K Resenberger, Markus Franke (277505), Waldron Sibeal, Jeremy C Simpson, Jörg Tatzelt (277508), James M. McCarthy (277504)   +31 more
core   +2 more sources

Immunomodulation for prion and prion-related diseases [PDF]

Expert Review of Vaccines, 2010
Prion diseases are a unique category of illness, affecting both animals and humans, where the underlying pathogenesis is related to a conformational change of a normal self protein called cellular prion protein to a pathological and infectious conformer known as scrapie prion protein (PrP(Sc)). Currently, all prion diseases lack effective treatment and
Thomas, Wisniewski, Fernando, Goñi
openaire   +2 more sources

Prion degradation pathways: Potential for therapeutic intervention [PDF]

, 2015
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
McKinnon, C, Tabrizi, SJ, Goold, R
core  

Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]

, 2010
Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Valerie Daggett, Daggett, Valerie, van der Kamp, Marc W, Marc W. van der Kamp   +3 more
core   +1 more source

Development of a sensitive cell culture system to assess prion infectivity and the efficacy of prion decontamination technologies

, 2012
Creutzfeldt-Jakob disease (CJD) can be iatrogenically transmitted during transplants, grafts and transfusions from CJD infected donors and also contaminated surgical instruments.
Secker, Thomas
core   +1 more source

PAD-Beads enrichment enhances detection of PrPSc using real-time quaking-induced conversion

BMC Research Notes, 2019
Objective Scrapie is a transmissible spongiform encephalopathy (TSE) that naturally occurs in sheep and goats. This fatal neurodegenerative disease results from misfolding of the normal cellular prion protein (PrPC) to a pathogenic prion protein form ...
Soyoun Hwang, Rohana P. Dassanayake, Eric M. Nicholson   +2 more
doaj   +1 more source