Results 51 to 60 of about 69,500 (311)

Posterior Cortical Atrophy in the Asia‐Pacific: A Report From the PCA Asian Workgroup

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Posterior Cortical Atrophy (PCA) is a distinct dementia syndrome primarily affecting spatial abilities and visual processing. It is associated with degeneration in the posterior part of the brain. PCA is subclassified into PCA‐pure and PCA‐plus syndromes based on consensus criteria.
Yuttachai Likitjaroen, Ya‐Ting Chang, Levinia Lim, Pai‐Yi Chiu, Jung‐Lung Hsu, SangYun Kim, Wataru Narita, Na Young Ryoo, Kyoko Suzuki, Nagaendran Kandiah, Sebastian Crutch, Ming‐Chyi Pai   +11 more
wiley   +1 more source

Estimating sequence diversity of prion protein gene (PRNP) in Portuguese populations of two cervid species: red deer and fallow deer

Prion, 2023
Among the transmissible spongiform encephalopathies (TSEs), chronic wasting disease (CWD) in cervids is now a rising concern in wildlife within Europe, after the detection of the first case in Norway in 2016, in a wild reindeer and until June 2022 a ...
Jorge C. Pereira, Nuno Gonçalves-Anjo, Leonor Orge, Maria A. Pires, Sara Rocha, Luís Figueira, Ana C. Matos, João Silva, Paula Mendonça, Paulo Carvalho, Paula Tavares, Carla Lima, Anabela Alves, Alexandra Esteves, Maria L. Pinto, Isabel Pires, Adelina Gama, Roberto Sargo, Filipe Silva, Fernanda Seixas, Madalena Vieira-Pinto, Estela Bastos   +21 more
doaj   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Vassallo, Neville, Herms, Jochen, Kretzschmar, Hans A.   +2 more
core  

SPG4 and Dementia: Expanding the Clinical Spectrum

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Hereditary spastic paraplegia (HSP) is a group of disorders characterized by progressive spasticity and lower limb weakness, with mutations in SPG4/SPAST being the most common cause. Detailed studies and clinical and molecular comparisons across different populations are missing.
Emanuele Panza, Arun Meyyazhagan, Eliseo Picchi, Gustavo Ribas, Ingrid Faber, Ryosuke Miyamoto, Preethi Basavaraju, Paolo Eusebi, Haripriya Kuchi Bhotla, Mario Stasi, Fabrizio Gaudiello, Francesco Patti, Filippo Maria Santorelli, Marcondes Cavalcante França Jr, José Luiz Pedroso, Orlando Graziani Povoas Barsottini, Hélio Afonso Ghizoni Teive, Peter Henry St George‐Hyslop, Toshitaka Kawarai, Antonio Orlacchio   +19 more
wiley   +1 more source

Variability in prion protein genotypes by spatial unit to inform susceptibility to chronic wasting disease

Prion, 2022
Chronic wasting disease (CWD) is a fatal encephalopathy affecting North American cervids. Certain alleles in a host’s prion protein gene are responsible for reduced susceptibility to CWD.
Alberto F. Fameli, Jessie Edson, Jeremiah E. Banfield, Christopher S. Rosenberry, W. David Walter   +4 more
doaj   +1 more source

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

Prions and Prion Diseases [PDF]

Laboratory Medicine, 1999
Prion diseases (transmissible encephalopathies) are progressive neurodegenerative diseases of animals and humans caused by a unique agent, the prion. These diseases have recently been in the news because of the emergence of bovine spongiform encephalopathy (BSE; mad cow disease) and variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom.
openaire   +1 more source

Molecular dynamics as an approach to study prion protein misfolding and the effect of pathogenic mutations [PDF]

, 2011
Computer simulation of protein dynamics offers unique high-resolution information that complements experiment. Using experimentally derived structures of the natively folded prion protein (PrP), physically realistic dynamics and conformational changes ...
Valerie Daggett, Daggett, Valerie, van der Kamp, Marc W, Marc W. van der Kamp   +3 more
core   +1 more source

Natural Biomaterials for Osteochondral Repair: From Source to Strategy

Advanced Healthcare Materials, EarlyView.
Biological origin‐guided overview of natural biomaterials and therapeutic strategies for osteochondral tissue engineering. The circular diagram categorizes representative materials and strategies into plant/algae‐derived, microbial‐derived, animal‐derived, and human‐derived sources, centered on an osteochondral defect repair model.
Hengyu Liu, Hanyang Zhang, Wenbo Yang, Hao Chen, Jincheng Wang, Fei Chang   +5 more
wiley   +1 more source

Characterisation and prion transmission study in mice with genetic reduction of sporadic Creutzfeldt-Jakob disease risk gene Stx6

Neurobiology of Disease
Sporadic Creutzfeldt-Jakob disease (sCJD), the most common human prion disease, is thought to occur when the cellular prion protein (PrPC) spontaneously misfolds and assembles into prion fibrils, culminating in fatal neurodegeneration.
Emma Jones, Elizabeth Hill, Jacqueline Linehan, Tamsin Nazari, Adam Caulder, Gemma F. Codner, Marie Hutchison, Matthew Mackenzie, Michael Farmer, Thomas Coysh, Michael Wiggins De Oliveira, Huda Al-Doujaily, Malin Sandberg, Emmanuelle Viré, Thomas J. Cunningham, Emmanuel A. Asante, Sebastian Brandner, John Collinge, Simon Mead   +18 more
doaj   +1 more source