Results 121 to 130 of about 33,090 (271)

A novel, resistance-linked ovine PrP variant and its equivalent mouse variant modulate the in vitro cell-free conversion of rPrP to PrPres

, 2006
Prion diseases are associated with the conversion of the normal cellular prion protein, PrPC, to the abnormal, disease-associated form, PrPSc. This conversion can be mimicked in vitro by using a cell-free conversion assay. It has recently been shown that
Kirby, Louise, Manson, Jean C., Houston, Fiona, Goldmann, Wilfred, Gill, Andrew C.   +4 more
core   +1 more source

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification [PDF]

, 2009
Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion ...
Maurizio Pocchiari, Roncaroli F, Cras, Patrick, Patrich Cras, Anna Ladogana, Giese, Armin, Cras, Patrich, Pocchiari M, Ladogana, Anna, Federico Roncaroli, Ladogana A, Bernardino Ghetti, Jan P. M. Langeveld, Strammiello, R., Zerr, I., Ghetti, B., Ghetti B, Roncaroli, Federico, Pocchiari, Maurizio, Langeveld, Jan P.M., Inga Zerr, Cras, P., Notari, Silvio, Cras P, Giese, A., Roncaroli, F., Parchi, Piero, Notari, S., Kretzschmar H, Langeveld JP, Rosaria Strammiello, Ghetti, Bernardino, Zerr, Inga, Kretzschmar, Hans A., Armin Giese, Parchi, P., Capellari, S., Hans Kretzschmar, Notari S, Pocchiari, M., Kretzschmar, Hans, Ladogana, A., Giese A, Langeveld, J.P.M., Capellari, Sabina, Strammiello, Rosaria, Piero Parchi, Silvio Notari, Zerr I, Langeveld, Jan P. M., Kretzschmar, H., Sabina Capellari   +51 more
core   +1 more source

Comments to the “Letter to the Editor” for the manuscript titled “Increased expression of inflammasome signaling genes and proteins in selective brain regions in the intermediate stage of Alzheimer's disease”

Brain Pathology, EarlyView.
Beta amyloid diffuse plaques, neurofibrillary tangles and neuritic plaques, are increased in densities at the intermediate stage of Alzheimer's neuropathological change. These pathological changes releasing Pathogen‐Associated Molecular Patterns (PAMPs) and Damage‐Associated Molecular Patterns (DAMPs).
Juan Pablo de Rivero Vaccari, David A. Davis, Andrew P. Sawaya, Susanna P. Garamszegi, Xiaoyan Sun, Ayled Barreda, Helen M. Bramlett, Sakir Humayun Gultekin, W. Dalton Dietrich, Robert W. Keane, Regina T. Vontell   +10 more
wiley   +1 more source

A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy [PDF]

, 1999
A mutation equivalent to P102L in the human PrP gene, associated with Gerstmann-Straussler syndrome (GSS), has been introduced into the murine PrP gene by gene targeting.
Ironside, James, Bostock, C, Somerville, Robert, Tuzi, N L, Baybutt, H, Hope, James, Manson, J C, McConnell, Irene, Baybutt, Herbert, Somerville, R, Ironside, J, Sy, M S, Jamieson, E, Tuzi, Nadia L., Melton, David W., McConnell, I, Will, R, Manson, Jean C., Sy, Man Sun, Melton, D W, Bostock, Christopher, Will, Robert, Sy, Man-Sun, Barron, Rona, Barron, R, Hope, J, Jamieson, Elizabeth   +26 more
core   +1 more source

MicroRNA Alterations in the Brain and Body Fluids of Humans and Animal Prion Disease Models: Current Status and Perspectives

Frontiers in Aging Neuroscience, 2018
Prion diseases are transmissible progressive neurodegenerative conditions characterized by rapid neuronal loss accompanied by a heterogeneous neuropathology, including spongiform degeneration, gliosis and protein aggregation.
Eirini Kanata, Katrin Thüne, Konstantinos Xanthopoulos, Isidre Ferrer, Isidre Ferrer, Isidre Ferrer, Dimitra Dafou, Inga Zerr, Theodoros Sklaviadis, Franc Llorens, Franc Llorens, Franc Llorens   +11 more
doaj   +1 more source

Proteostasis of organelles in aging and disease

The FEBS Journal, EarlyView.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

Molecular dynamics simulations elucidate the misfolding mechanisms of secretion‐defective pancreatic lipase variants

The FEBS Journal, EarlyView.
Misfolding mutations in pancreatic lipase have been identified as potential contributors of chronic pancreatitis, an inflammatory disease of the human pancreas. Here, we describe the effect of these misfolding mutations on pancreatic lipase structure using molecular dynamics simulations and structural modeling.
Gyula Hoffka, András Szabó
wiley   +1 more source

Endogenous Viral Etiology of Prion Diseases

, 2009
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of incurable neurodegenerative disorders, including Kuru and Creutzfeldt-Jakob disease in humans, “mad cow” disease in cattle, and scrapie in sheep. This paper
Claudiu I. Bandea
core  

Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt-Jakob disease

, 2005
In recent studies, the amyloid form of recombinant prion protein (PrP) encompassing residues 89-230 (rPrP 89-230) produced in vitro induced transmissible prion disease in mice.
Bocharova, O V, Salnikov, V V, Gill, A C, Vadim V. Salnikov, Ilia V. Baskakov, Leonid Breydo, Breydo, L, Olga V. Bocharova, Andrew C. Gill, Baskakov, I V   +9 more
core   +1 more source

Bone Grafts: Everything You Need to Know

Journal of Periodontal Research, EarlyView.
This review classifies bone grafts (autograft, allograft, xenograft, synthetic) by source and osteogenic/inductive/conductive potential, linking material properties and mechanisms to clinical indications in periodontal and dental regeneration. Key challenges—limited osteoinduction, poor vascularisation, unbalanced resorption, handling difficulties, and
Håvard Jostein Haugen, Javier Sanz, Giuseppe Perale, Augustine Mark Saiz, Mario Romandini, Maryam Rahmati   +5 more
wiley   +1 more source