Results 21 to 30 of about 4,898,785 (204)

Prion Diseases [PDF]

Seminars in Neurology, 2012
Prion diseases are a group of diseases caused by abnormally conformed infectious proteins, called prions. They can be sporadic (Jakob-Creutzfeldt disease [JCD]), genetic (genetic JCD, Gerstmann-Sträussler-Scheinker, and familial fatal insomnia), or acquired (kuru, variant JCD, and iatrogenic JCD).
Takada, Leonel, Geschwind, Michael
openaire   +4 more sources

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

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

Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles. [PDF]

, 2006
Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model.
Bari Michele A. Di, Boyle, Aileen, Lipp Hans-Peter, Gabriele Vaccari, Maurizio Pocchiari, Cardone Franco, Sbriccoli Marco, Claudia Cartoni, Sbriccoli, Marco, Boyle Aileen, Dell'Omo, Giacomo, Nonno, Romolo, Di Bari, Michele A, Nonno Romolo, Franco Cardone, Galeno, Roberta, Marco Sbriccoli, Paola Fazzi, Vaccari Gabriele, Cartoni, Claudia, Cardone, Franco, Galeno Roberta, Loredana Ingrosso, Roberta Galeno, Fazzi, Paola, Lipp, Hans-Peter, Fazzi Paola, Michele A Di Bari, Ingrosso Loredana, Hans-Peter Lipp, Ingrosso, Loredana, Pocchiari, Maurizio, Umberto Agrimi, Pocchiari Maurizio, Vaccari, Gabriele, Agrimi, Umberto, Giacomo Dell'Omo, Moira Bruce, Aileen Boyle, Dell'Omo Giacomo, Agrimi Umberto, Bruce Moira, Bruce, Moira, Romolo Nonno, Cartoni Claudia   +44 more
core   +1 more source

Characterization of the prion protein in relation to normal cellular function and in disease [PDF]

, 2012
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of rare and fatal neurodegenerative disorders that can affect both human and animals.
Wik, Lotta
core   +1 more source

A Retrospective Cohort Study of a Newly Proposed Criteria for Sporadic Creutzfeldt–Jakob Disease

Diagnostics
Background/Objectives: Sporadic Creutzfeldt–Jakob disease (sCJD) is a fatal neurodegenerative disorder traditionally diagnosed based on the World Health Organization (WHO) criteria in 1998. Recently, Hermann et al.
Toshiaki Nonaka, Ryusuke Ae, Koki Kosami, Hiroya Tange, Miho Kaneko, Takehiro Nakagaki, Tsuyoshi Hamaguchi, Nobuo Sanjo, Yoshikazu Nakamura, Tetsuyuki Kitamoto, Yoshiyuki Kuroiwa, Kensaku Kasuga, Manabu Doyu, Fumiaki Tanaka, Koji Abe, Shigeo Murayama, Ichiro Yabe, Hideki Mochizuki, Takuya Matsushita, Hiroyuki Murai, Masashi Aoki, Koji Fujita, Masafumi Harada, Masaki Takao, Tadashi Tsukamoto, Yasushi Iwasaki, Masahito Yamada, Hidehiro Mizusawa, Katsuya Satoh, Noriyuki Nishida   +29 more
doaj   +1 more source

Effects of post-translational modifications on prion protein aggregation and the propagation of scrapie-like characteristics in vitro [PDF]

, 2007
Prion diseases, or transmissible spongiform encephalopathies (TSEs) are typically characterised by CNS accumulation of PrPSc, an aberrant conformer of a normal cellular protein PrPC.
Oxley, David, Meersman, Filip, Webster, Judith, Kazlauskaite, Jurate, Pinheiro, Teresa J. T., Young, Duncan S., Dear, Denise V., Lowe, Christopher R., Gill, Andrew C., Bronstein, Igor   +9 more
core   +1 more source

A novel protective prion protein variant that colocalizes with kuru exposure. [PDF]

, 2009
BACKGROUND: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes.
Whittaker, John, Mein, C., Shah, Paresh, Campbell, T., Whitfield, Jerome, Mein, Charles A, Whittaker, J., Alpers, Michael Philip, Hummerich, H., Collinge, J., Hummerich, Holger, Collinge, John, Uphill, J., Poulter, Mark, Beck, Jon, Al-Dujaily, Huda, Mead, S., Al-Dujaily, H., Alpers, Michael P, Beck, J., Verzilli, C., Mead, Simon, Verzilli, Claudio, Shah, P., Uphill, James, Poulter, M., Campbell, Tracy   +26 more
core   +1 more source

A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody

Scientific Reports, 2022
There is an urgent need to develop disease-modifying therapies to treat neurodegenerative diseases which pose increasing challenges to global healthcare systems.
Madeleine Reilly, Iryna Benilova, Azadeh Khalili-Shirazi, Christian Schmidt, Parvin Ahmed, Daniel Yip, Parmjit S. Jat, John Collinge   +7 more
doaj   +1 more source

Genetic aspects of human prion diseases

Frontiers in Neurology, 2022
Human prion diseases are rapidly progressive and fatal neurodegenerative conditions caused by a disease-causing isoform of the native prion protein. The prion protein gene (PRNP) encodes for the cellular prion protein, which is the biological substrate ...
Brian S. Appleby, Brian S. Appleby, Shashirekha Shetty, Shashirekha Shetty, Mohamed Elkasaby   +4 more
doaj   +1 more source