Results 61 to 70 of about 8,574 (264)

The 37kDa/67kDa laminin receptor as a therapeutic target in prion diseases: potency of antisense LRP RNA, siRNAs specific for LRP mRNA and a LRP decoy mutant [PDF]

, 2006
Prion diseases are a group of rare, fatal neurodegenerative diseases, also known as transmissible spongiform encephalopathies (TSEs), that affect both animals and humans and include bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep ...
Vana, Karen
core   +1 more source

Chronic Wasting Disease in Farmed Cervids, South Korea, 2001–2024

Emerging Infectious Diseases
Chronic wasting disease (CWD) was identified in imported elk in South Korea in 2001 and has spread among cervids nationwide. The country’s surveillance and control policy culls cervids from any CWD-positive farms, and prevalence during 2020–2024 was
Young Pyo Choi, Yu-Ran Lee, Hoo Chang Park, Yoon Hee Lee, Gordon Mitchell, In-Soon Roh, Hyun-Joo Sohn   +6 more
doaj   +1 more source

Reflections on Cerebellar Neuropathology in Classical Scrapie

Biomolecules, 2021
In this review, the most important neuropathological changes found in the cerebella of sheep affected by classical natural scrapie are discussed. This disease is the oldest known of a group of unconventional “infections” caused by toxic prions of ...
Adolfo Toledano-Díaz, María Isabel Álvarez, Jose-Julio Rodríguez, Juan Jose Badiola, Marta Monzón, Adolfo Toledano   +5 more
doaj   +1 more source

Pros and cons of a prion-like pathogenesis in Parkinson's disease [PDF]

, 2011
Background: Parkinson's disease (PD) is a slowly progressive neurodegenerative disorder which affects widespread areas of the brainstem, basal ganglia and cerebral cortex.
Chapman Joab, Hilker, Ruediger, Brotchie, Jonathan M, Hilker, Rüdiger, Hilker Ruediger, Brotchie, Jonathan M., Joab Chapman, Ruediger Hilker, Chapman, Joab, Jonathan M Brotchie, Brotchie Jonathan M   +10 more
core   +2 more sources

Structural Polymorphism of polyG Inclusions Revealed by In Situ Cryo‐Electron Tomography

Advanced Science, EarlyView.
Correlative cryo‐electron tomography in primary cortical neurons and NIID mouse brain tissue reveals that polyG inclusions are interconnected ribbon‐like assemblies rather than canonical amyloid fibrils. Multiple compartment‐specific ribbon states show distinct 26S proteasome accessibility, while cytoplasmic ribbons contact and deform ER‐like ...
Yunwen Qian, Yalan Wan, Chen Chen, Xiaoyu Zheng, Wenjing Du, Junhan Yang, Zhihao Quan, Biyu Yang, Jiaxi Yu, Jie Zheng, Zhaoxia Wang, Jianwen Deng, Qiang Guo   +12 more
wiley   +1 more source

Endogenous Viral Etiology of Prion Diseases [PDF]

, 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  

A Phosphorylation‐Induced Micellization Switch in the Low‐Complexity Domain of TDP‐43

Advanced Science, EarlyView.
Phosphorylation of TAR DNA‐binding protein's 43 kDa (TDP‐43) low‐complexity domain by casein kinase 1 delta (CK1δ) acts as a molecular switch, redirecting its self‐assembly from macroscopic phase separation toward finite‐sized, spherical block‐copolymer micelles of ∼30 nm.
Rodrigo F. Dillenburg, Anastasia Lopatina, Hao Ruan, Tom Scheidt, Simone Mosna, Emre Pekbilir, Julia Bieber, Frank Schäfer‐Depoix, Katharina Landfester, Carla Schmidt, Martin M. Möckel, Svenja Morsbach, Friederike Schmid, Dorothee Dormann, Lukas Stelzl, Martin Girard, Edward A. Lemke   +16 more
wiley   +1 more source

Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease

Emerging Infectious Diseases, 2012
Chronic wasting disease (CWD) is a fatal, transmissible prion disease that affects captive and free-ranging deer, elk, and moose. Although the zoonotic potential of CWD is considered low, identification of multiple CWD strains and the potential for agent
Samuel E. Saunders, Shannon L. Bartelt-Hunt, Jason C. Bartz   +2 more
doaj   +1 more source

PolyG Fibrils Coalesce Into Nuclear Ribbons That Engage Proteostasis Machinery in Neuronal Intranuclear Inclusion Disease

Advanced Science, EarlyView.
In NIID, expanded NOTCH2NLC repeats give rise to nuclear polyG inclusions. Tracer‐guided in situ cryo‐electron tomography enables cross‐scale structural analysis from mouse brain to native neuronal nuclei, revealing dense‐core/peripheral‐halo inclusions built from compact polyG ribbons.
Hui Dong, Yongcheng Pan, Zhiyao Tang, Yuxuan Yao, Guicong Zhang, Junpu Wang, Haonan Xiao, Yun Tian, Beisha Tang, Dan Li, Qiang Guo, Ruijun Tian, Qiong Liu, Cong Liu   +13 more
wiley   +1 more source

Prions and Public Health Office (PPHO) [PDF]

PPHO monitors human prion diseases in the US and coordinates programs related to these and other selected neurological diseases and illnesses of unknown causes such as Nodding syndrome and Kawasaki syndrome.

core   +2 more sources