Results 71 to 80 of about 7,097 (179)

A receptor for infectious and cellular prion protein

Brazilian Journal of Medical and Biological Research, 1999
Prions are an unconventional form of infectious agents composed only of protein and involved in transmissible spongiform encephalopathies in humans and animals.
V.R. Martins
doaj   +1 more source

Peripheral Signatures of Multidimensional Pathology in Symptomatic and Asymptomatic Creutzfeldt–Jakob Disease

CNS Neuroscience &Therapeutics, Volume 32, Issue 1, January 2026.
Plasma biomarkers in CJD reveal multisystem involvement, with neuronal injury markers showing strong discriminative performance and vascular proteins indicating blood–brain barrier dysfunction. In asymptomatic PRNP mutation carriers, biomarker changes are minimal and emerge mainly near disease onset ABSTRACT Background Plasma markers of neuronal injury
Zhong‐Yun Chen, Min Chu, Yi‐Hao Wang, Rui Liu, Jing Zhang, Ai‐ling Yue, Hua Lu, Qian‐qian He, Jia‐hui Hou, Yu‐fei Chen, Hong Ye, Li‐Yong Wu   +11 more
wiley   +1 more source

Exploration of the main sites for the transformation of normal prion protein (PrPC) into pathogenic prion protein (PrPsc)

Journal of Veterinary Research, 2017
Introduction: The functions and mechanisms of prion proteins (PrPC) are currently unknown, but most experts believe that deformed or pathogenic prion proteins (PrPSc) originate from PrPC, and that there may be plural main sites for the conversion of ...
Liu Xi-Lin, Feng Xiao-Li, Wang Guang-Ming, Gong Bin-Bin, Ahmad Waqas, Liu Nan-Nan, Zhang Yuan-Yuan, Yang Li, Ren Hong-Lin, Cui Shu-Sen   +9 more
doaj   +1 more source

Evaluation of Confocal Fluorescence Spectroscopy for the Detection of Pathological Prion Proteins

CHIMIA, 2005
Prion diseases or transmissible spongiform encephalopathies (TSEs) are characterized by the accumulation in the brain of PrPSc, an abnormal isoform of the host-encoded glycoprotein PrPC.
Anja Dietrich, Karin Bossart, Bruno Oesch, Christoph Stamm, Alex J. Raeber   +4 more
doaj   +1 more source

Pathologic and biochemical characterization of PrPSc from elk with PRNP polymorphisms at codon 132 after experimental infection with the chronic wasting disease agent

BMC Veterinary Research, 2018
Background The Rocky Mountain elk (Cervus elaphus nelsoni) prion protein gene (PRNP) is polymorphic at codon 132, with leucine (L132) and methionine (M132) allelic variants present in the population.
S. Jo Moore, Catherine E. Vrentas, Soyoun Hwang, M. Heather West Greenlee, Eric M. Nicholson, Justin J. Greenlee   +5 more
doaj   +1 more source

Cofactor and glycosylation preferences for in vitro prion conversion are predominantly determined by strain conformation.

PLoS Pathogens, 2020
Prion diseases are caused by the misfolding of a host-encoded glycoprotein, PrPC, into a pathogenic conformer, PrPSc. Infectious prions can exist as different strains, composed of unique conformations of PrPSc that generate strain-specific biological ...
Cassandra M Burke, Daniel J Walsh, Kenneth M K Mark, Nathan R Deleault, Koren A Nishina, Umberto Agrimi, Michele A Di Bari, Surachai Supattapone   +7 more
doaj   +1 more source

Atypical Creutzfeldt-Jakob disease with PrP-amyloid plaques in white matter: molecular characterization and transmission to bank voles show the M1 strain signature

Acta Neuropathologica Communications, 2017
Amyloid plaques formed by abnormal prion protein (PrPSc) aggregates occur with low frequency in Creutzfeldt-Jakob disease, but represent a pathological hallmark of three relatively rare disease histotypes, namely variant CJD, sporadic CJDMV2K (methionine/
Marcello Rossi, Daniela Saverioni, Michele Di Bari, Simone Baiardi, Afina Willemina Lemstra, Laura Pirisinu, Sabina Capellari, Annemieke Rozemuller, Romolo Nonno, Piero Parchi   +9 more
doaj   +1 more source

A novel approach for scrapie-associated prion (PrPSc) detection in blood using the competitive affinity of an aggregate-specific antibody and streptavidin to PrPSc

Research in Veterinary Science, 2017
Scrapie is a fatal neurodegenerative disorder affecting sheep and goats, originating from exposure to disease-associated prions (PrPSc). An ante-mortem screening test that can detect native PrPSc in body fluids remains unavailable due to insufficient sensitivity of current detection methods that involve proteinase or denaturation treatments. We adopted
Andrei, Soutyrine, Hongsheng, Huang, Olga, Andrievskaia, Ines, Walther, Gordon, Mitchell   +4 more
openaire   +2 more sources

Involvement of Dab1 in APP processing and β-amyloid deposition in sporadic Creutzfeldt–Jakob patients

Neurobiology of Disease, 2010
Alzheimer's disease and prion pathologies (e.g., Creutzfeldt–Jakob disease (CJD)) display profound neural lesions associated with aberrant protein processing and extracellular amyloid deposits.
R. Gavín, I. Ferrer, J.A. del Río
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Altered distribution, aggregation, and protease resistance of cellular prion protein following intracranial inoculation.

PLoS ONE, 2019
Prion protein (PrPC) is a protease-sensitive and soluble cell surface glycoprotein expressed in almost all mammalian cell types. PrPSc, a protease-resistant and insoluble form of PrPC, is the causative agent of prion diseases, fatal and transmissible ...
Anne Ward, Jason R Hollister, Young Pyo Choi, Brent Race, Katie Williams, Daniel W Shoup, Roger A Moore, Suzette A Priola   +7 more
doaj   +1 more source