Results 21 to 30 of about 8,574 (264)

Molecular Nanobiotechnological Approaches for the Detection and Therapy of Prion Related Diseases [PDF]

Nano Biomedicine and Engineering, 2012
Prion diseases are associated with the accumulation in the brain of an abnormal, protease resistant isoform of a host encoded glycoprotein known as prion protein (PrP). Nanotechnology in combination with biotechniques promises a broad spectrum of highly innovative approaches for overcoming the challenges posed by the prions.
PK Praseetha, Anand S Thampy, P Venugopalan, Murthy S Chavali   +3 more
openaire   +2 more sources

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

Transmission Characteristics of Variably Protease-Sensitive Prionopathy

Emerging Infectious Diseases, 2014
Variably protease-sensitive prionopathy (VPSPr), a recently identified and seemingly sporadic human prion disease, is distinct from Creutzfeldt-Jakob disease (CJD) but shares features of Gerstmann-Sträussler-Scheinker disease (GSS).
Silvio Notari, Xiangzhu Xiao, Juan Carlos Espinosa, Yvonne Cohen, Liuting Qing, Patricia Aguilar-Calvo, Diane Kofskey, Ignazio Cali, Laura Cracco, Qingzhong Kong, Juan Maria Torres, Pierluigi Gambetti   +11 more
doaj   +1 more source

Defining the Protein Seeds of Neurodegeneration using Real-Time Quaking-Induced Conversion Assays

Biomolecules, 2020
Neurodegenerative diseases are characterized by the accumulation of disease-related misfolded proteins. It is now widely understood that the characteristic self-amplifying (i.e., seeding) capacity once only attributed to the prions of transmissible ...
Matteo Manca, Allison Kraus
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

Experimental Oronasal Transmission of Chronic Wasting Disease Agent from White-Tailed Deer to Suffolk Sheep

Emerging Infectious Diseases, 2021
Chronic wasting disease (CWD) is a fatal prion disease of cervids. We examined host range of CWD by oronasally inoculating Suffolk sheep with brain homogenate from a CWD-positive white-tailed deer.
Eric D. Cassmann, S. Jo Moore, Justin J. Greenlee   +2 more
doaj   +1 more source

Can loss-of-function prion-related diseases exist? [PDF]

Molecular Psychiatry, 1998
Discovery of mutations of the PrnP gene without typical plaque formation and the characterization of two prion receptors led us to postulate a new class of prion-related disease: ‘loss of function’.
H B Samaia, R R Brentani
openaire   +1 more source

Insufficient hydrogen‐bond desolvation and prion‐related disease [PDF]

European Journal of Biochemistry, 2002
A structuring and eventual exclusion of water surrounding backbone hydrogen bonds takes place during protein folding as hydrophobic residues cluster around such bonds. Taken as an average over all hydrogen bonds, the extent of desolvation is nearly a constant of motion, as revealed by re‐examination of the longest all‐atom trajectory with explicit ...
openaire   +2 more sources

Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients

Emerging Infectious Diseases, 2017
In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities.
Jean Y. Douet, Caroline Lacroux, Naima Aron, Mark W. Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W. Ironside, Olivier Andréoletti   +11 more
doaj   +1 more source

THERPA v2: an update of a small molecule database related to prion protein regulation and prion disease progression [PDF]

Prion, 2019
Prion diseases are rare, rapidly progressive neurodegenerative disorders that affect mammalian species [1,2].
Sol Moe Lee, Sung Soon Kim, Heebal Kim, Su Yeon Kim   +3 more
openaire   +2 more sources