Results 71 to 80 of about 1,598,142 (264)

Comprehensive Characterization of 98 Chinese Cases of Genetic Creutzfeldt‐Jakob Disease With T188K Mutation

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To characterize the demographic, clinical, and laboratory features of the Chinese patients of genetic Creutzfeldt‐Jakob disease with T188K variant (T188K‐gCJD), the most common subtype of genetic prion diseases (gPrDs) in China. Methods In this nationwide retrospective study, data from 98 genetically confirmed T188K‐gCJD patients ...
Chun‐Jie Li, Dong‐Lin Liang, Li‐Ping Gao, Wei‐Wei Zhang, Wei Zhou, A. Ruhan, Bing Xu, Run‐Dong Cao, Kang Xiao, Cao Chen, Qi Shi, Xiao‐Ping Dong   +11 more
wiley   +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

Preserving Proton Resolution Under Cryogenic Dynamic Nuclear Polarization for Biomolecular Solid‐State NMR

Angewandte Chemie, EarlyView.
High‐resolution proton detection is preserved under cryogenic dynamic nuclear polarization (DNP) by combining fast MAS, extensive deuteration and selective methyl protonation. This strategy enables proton‐detected structural studies of biomolecular solids with enhanced sensitivity under DNP conditions.
James Tolchard, Tanguy Le Marchand, Nadia El Mammeri, Kshama Sharma, Ajit Kumar Bishoyi, Mélanie Berbon, Kristaps Jaudzems, Dorothea Wisser, Asen Daskalov, Sven J. Saupe, Jan Stanek, Zhiyu Sun, Olivier Ouari, Birgit Habenstein, Anne Lesage, Guido Pintacuda, Antoine Loquet   +16 more
wiley   +2 more sources

Increasing prion propensity by hydrophobic insertion.

PLoS ONE, 2014
Prion formation involves the conversion of proteins from a soluble form into an infectious amyloid form. Most yeast prion proteins contain glutamine/asparagine-rich regions that are responsible for prion aggregation.
Aaron C Gonzalez Nelson, Kacy R Paul, Michelina Petri, Noe Flores, Ryan A Rogge, Sean M Cascarina, Eric D Ross   +6 more
doaj   +1 more source

Characterizing Cutaneous α‐Synuclein Deposition and Seeding Activity in Parkinson's Disease Subtypes

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Cutaneous phosphorylated α‐synuclein (p‐syn) and α‐synuclein seeding activity are promising biomarkers for Parkinson's disease (PD), but their clinical value remains uncertain due to disease heterogeneity. This study evaluates these two biomarkers in PD patients to inform phenotype‐specific diagnosis and disease severity assessment ...
Yuting Jin, Tianyuan Yang, Minglei Liu, Haiyang Luo, Jing Yuan, Ke Zhang, Xinsheng Han, Jing Yang, Yuming Xu   +8 more
wiley   +1 more source

Prion protein E219K polymorphism: from the discovery of the KANNO blood group to interventions for human prion disease

Frontiers in Neurology
KANNO is a new human blood group that was recently discovered. The KANNO antigen shares the PRNP gene with the prion protein and the prion protein E219K polymorphism determines the presence or absence of the KANNO antigen and the development of anti ...
Si-Si Wang, Zhao-Li Meng, Yi-Wen Zhang, Yi-Shuang Yan, Ling-Bo Li   +4 more
doaj   +1 more source

Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases

Frontiers in Cellular Neuroscience, 2019
Histopathological studies revealed that progressive neuropathies including Alzheimer, and Prion diseases among others, include accumulations of misfolded proteins intracellularly, extracellularly, or both.
Emiliano Zamponi, Gustavo F. Pigino
doaj   +1 more source

Prion protein—Semisynthetic prion protein (PrP) variants with posttranslational modifications [PDF]

Journal of Peptide Science, 2019
Deciphering the pathophysiologic events in prion diseases is challenging, and the role of posttranslational modifications (PTMs) such as glypidation and glycosylation remains elusive due to the lack of homogeneous protein preparations. So far, experimental studies have been limited in directly analyzing the earliest events of the conformational change ...
Stefanie Hackl, Christian F.W. Becker
openaire   +4 more sources

Stage‐Dependent β‐Synuclein Links MRI and Cognitive Decline in Alzheimer's Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Synaptic degeneration drives cognitive decline in Alzheimer's disease (AD), but synaptic biomarkers are scarce. Brain‐enriched β‐synuclein emerged as a synaptic damage marker. We investigated its diagnostic, prognostic, and structural correlates across the AD continuum.
Ulaş Ay, Merve Alaylioglu, Erdi Sahin, Sanem Sultan Yoruk Oner, Gulce Iskan, Gulce Cosku Yilmaz Cakan, Gozde Kizilates‐Evin, Emre Hari, Elif Kurt, Erdinc Dursun, Basar Bilgic, Hasmet Hanagasi, Hakan Gurvit, Tamer Demiralp, Duygu Gezen‐Ak, Bedia Samanci   +15 more
wiley   +1 more source

Effect of enzymatic deimination on the conformation of recombinant prion protein [PDF]

, 2009
Deimination is the post-translational conversion of arginine residues to citrulline. It has been implicated as a causative factor in autoimmune diseases such as multiple sclerosis and rheumatoid arthritis and more recently, as a marker of ...
Oxley, David, Meersman, Filip, Webster, Judith, Young, Duncan S., Dear, Denise V., Bronstein, Igor, Gill, Andy   +6 more
core