Results 81 to 90 of about 616,892 (193)

Association between CSF alpha-synuclein seeding activity and genetic status in Parkinson’s disease and dementia with Lewy bodies [PDF]

, 2021
The clinicopathological heterogeneity in Lewy-body diseases (LBD) highlights the need for pathology-driven biomarkers in-vivo. Misfolded alpha-synuclein (α-Syn) is a lead candidate based on its crucial role in disease pathophysiology.
Baiardi, S, Blennow, K, Brinkmalm, A, Brockmann, K, Deuschle, C, Gasser, T, Hauser, AK, Lachmann, I, Lerche, S, Mammana, A, Parchi, P, Quadalti, C, Roeben, B, Rossi, M, Schulte, C, Sjödin, S, Waniek, K, Wurster, I, Zetterberg, H, Zimmermann, M   +19 more
core   +1 more source

Skin RT-QuIC Assays are More Sensitive than CSF RT-QuIC in Prion Detection for Chinese Probable Sporadic Creutzfeldt-Jakob Disease

, 2020
Abstract BackgroundThe definite diagnosis of human sporadic Creutzfeldt-Jakob disease (sCJD) largely depends on postmortem neuropathology and PrPSc detection in the brain. The development of prion RT-QuIC of cerebrospinal fluid (CSF) samples makes it possible for premortem diagnosis for sCJD.
Kang Xiao, Xue-Hua Yang, Wei Zhou, Cao Chen, Brian S Appleby, Wen-Quan Zou, XiaoPing Dong, Qi Shi   +7 more
openaire   +1 more source

Real time-quaking induced conversion assay (RT-QUIC) v1

, 2022
Seeded-amplification assay (SAA) method for the detection of pathology-associated aggregation-templating competent forms of alpha-synuclein and other interacting factors.
andrew.west west, Arpine Sokratian
openaire   +1 more source

Effect of the micro-environment on alpha-synuclein conversion and implication in seeded conversion assays [PDF]

, 2020
Background: α-Synuclein is a small soluble protein, whose physiological function in the healthy brain is poorly understood. Intracellular inclusions of α-synuclein, referred to as Lewy bodies (LBs), are pathological hallmarks of α- synucleinopathies ...
Candelise, Niccolo, Cramm, Maria, Llorens Torres, Franc, Rabano, Alberto, Schmitz, Matthias, Stoops, Erik, Thüne, Katrin, Vanderstichele, Hugo, Villar Piqué, Anna, Zafar, Saima, Zerr, Inga   +10 more
core   +1 more source

RT-QuIC Using C-Terminally Truncated α-Synuclein Forms Detects Differences in Seeding Propensity of Different Brain Regions from Synucleinopathies

Biomolecules, 2021
Aggregated α-synuclein (αSyn) protein is a core pathological feature of Parkinson’s disease (PD) and dementia with Lewy bodies (DLB). Both PD and DLB demonstrate the presence of diverse intracellular α-synuclein (αSyn) species, including C-terminally ...
Ilaria Poggiolini, Daniel Erskine, Nishant N. Vaikath, Janarthanan Ponraj, Said Mansour, Christopher M. Morris, Omar M. A. El-Agnaf   +6 more
doaj   +1 more source

A noninvasive test for human prion disease using hair roots and scalp

Scientific Reports
Invasive tests like cerebrospinal fluid (CSF) examination are highly effective for diagnosing human prion disease (HPD). Real-time quaking-induced conversion (RT-QuIC) CSF assay demonstrates 80–90% sensitivity for HPD diagnosis.
Thi-Thu-Trang Dong, Hiroyuki Honda, Akio Akagi, Yasushi Iwasaki, Hitaru Kishida, Tadashi Tsukamoto, Kensaku Kasuga, Hirotsugu Takashima, Tatsuhiro Terada, Kensuke Ikenaka, Takeshi Ikeuchi, Tsuyoshi Mori, Hideki Mochizuki, Kenjiro Ono, Yoshihisa Takiyama, Tsuyoshi Hamaguchi, Hiroyuki Murota, Nobuo Sanjo, Takeshi Fujimoto, Michio Kitayama, Koji Fujita, Motohiro Yukitake, Shinsuke Fujioka, Noriyuki Nishida, Yoshio Tsuboi, Tetsuyuki Kitamoto, Masaki Takao, Masahito Yamada, Hidehiro Mizusawa, Katsuya Satoh   +29 more
doaj   +1 more source

Nanoplastics and Neurodegeneration: A Roadmap From Mechanism to Causation

Advanced Science, EarlyView.
Nanoplastics are pervasive environmental contaminants with potentially profound implications for human health. Emerging evidence suggests a possible link between nanoplastic exposure and neurodegeneration, a key driver of ageing and dementia, yet causality remains unresolved.
Yuhuan Li, Yue Wang, Xiufang Liang, Mengze Xu, David T. Leong, Pu Chun Ke   +5 more
wiley   +1 more source

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

Prion disease: experimental models and reality [PDF]

, 2017
The understanding of the pathogenesis and mechanisms of diseases requires a multidisciplinary approach, involving clinical observation, correlation to pathological processes, and modelling of disease mechanisms.
Brandner, S, Jaunmuktane, Z
core   +1 more source

The L108I polymorphism in mouse prion protein drives spontaneous disease and enhances transmission of atypical and classical prion strains

Brain Pathology, EarlyView.
A single amino acid change (L108I) combined with PrP overexpression drives spontaneous atypical prion formation in mice, enabling also efficient propagation of diverse prion strains. This model allows studying how spontaneous prion diseases arise and provides powerful tools for investigating strain emergence, transmission barriers, and mechanisms ...
Hasier Eraña, Enric Vidal, Natalia Fernández‐Borges, Jorge M. Charco, Carlos M. Díaz‐Domínguez, Cristina Sampedro‐Torres‐Quevedo, Josu Galarza‐Ahumada, Eva Fernández‐Muñoz, Maitena San‐Juan‐Ansoleaga, Miguel Ángel Pérez‐Castro, Nuno Gonçalves‐Anjo, Patricia Piñeiro, Samanta Giler, Nora González‐Martín, Nuria L. Lorenzo, Africa Manero‐Azua, Guiomar Perez de Nanclares, Mariví Geijo, Manuel A. Sánchez‐Martín, Jesús R. Requena, Joaquín Castilla   +20 more
wiley   +1 more source