Acta Neuropathologica Communications

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Brain tumor with an ATXN1-NUTM1 fusion gene expands the histologic spectrum of NUTM1-rearranged neoplasia
Acta Neuropathologica Communications - Tập 7 - Trang 1-3 - 2019
Aurore Siegfried, Julien Masliah-Planchon, Franck-Emmanuel Roux, Delphine Larrieu-Ciron, Gaelle Pierron, Yvan Nicaise, Marion Gambart, Isabelle Catalaa, Sarah Péricart, Charlotte Dubucs, Badreddine Mohand-Oumoussa, Franck Tirode, Franck Bourdeaut, Emmanuelle Uro-Coste
Cerebral organoids: emerging ex vivo humanoid models of glioblastoma
Acta Neuropathologica Communications - Tập 8 - Trang 1-9 - 2020
Michail-Dimitrios Papaioannou, Kevin Sangster, Rifat Shahriar Sajid, Ugljesa Djuric, Phedias Diamandis
Glioblastoma is an aggressive form of brain cancer that has seen only marginal improvements in its bleak survival outlook of 12–15 months over the last forty years. There is therefore an urgent need for the development of advanced drug screening platforms and systems that can better recapitulate glioblastoma’s infiltrative biology, a process largely responsible for its relentless propensity for re...... hiện toàn bộ
CNS-associated macrophages contribute to intracerebral aneurysm pathophysiology
Acta Neuropathologica Communications - Tập 12 Số 1
Martina Glavan, Andrea Jelić, Damien Levard, Juhana Frösén, Sara Keränen, Bart A. A. Franx, Ana Rita Brás, Estelle R Louet, Ádám Dénes, Mario Merlini, Denis Vivien, Marina Rubio
AbstractIntracerebral aneurysms (IAs) are pathological dilatations of cerebral arteries whose rupture leads to subarachnoid hemorrhage, a significant cause of disability and death. Inflammation is recognized as a critical contributor to the formation, growth, and rupture of IAs; however, its precise actors have not yet been fully elucidated. Here, we report CNS-ass...... hiện toàn bộ
α-Synuclein pathology in Parkinson disease activates homeostatic NRF2 anti-oxidant response
Acta Neuropathologica Communications - Tập 9 - Trang 1-16 - 2021
Alberto Delaidelli, Mette Richner, Lixiang Jiang, Amelia van der Laan, Ida Bergholdt Jul Christiansen, Nelson Ferreira, Jens R. Nyengaard, Christian B. Vægter, Poul H. Jensen, Ian R. Mackenzie, Poul H. Sorensen, Asad Jan
Circumstantial evidence points to a pathological role of alpha-synuclein (aSyn; gene symbol SNCA), conferred by aSyn misfolding and aggregation, in Parkinson disease (PD) and related synucleinopathies. Several findings in experimental models implicate perturbations in the tissue homeostatic mechanisms triggered by pathological aSyn accumulation, including impaired redox homeostasis, as significant...... hiện toàn bộ
New somatic TERT promoter variants enhance the Telomerase activity in Glioblastoma
Acta Neuropathologica Communications - Tập 8 - Trang 1-10 - 2020
Tiziana Pierini, Carlotta Nardelli, Anair Graciela Lema Fernandez, Valentina Pierini, Fabrizia Pellanera, Valeria Nofrini, Paolo Gorello, Martina Moretti, Silvia Arniani, Giovanni Roti, Paolo Giovenali, Marco Lupattelli, Giulio Metro, Carmen Molica, Corrado Castrioto, Rodolfo Corinaldesi, Maria Elena Laurenti, Stefano Ascani, Cristina Mecucci, Roberta La Starza
The catalytic activity of human Telomerase Reverse Transcriptase (TERT) compensates for the loss of telomere length, eroded during each cell cycle, to ensure a correct division of stem and germinal cells. In human tumors, ectopic TERT reactivation, most frequently due to hotspot mutations in the promoter region (TERTp), i.e. c.1-124 C > T, c.1-146 C > T, confers a proliferative advantage to neopla...... hiện toàn bộ
PrPres deposition in the retina is a common finding of sporadic, familial and iatrogenic Creutzfeldt-Jakob diseases (CJD)
Acta Neuropathologica Communications - - 2018
Masaki Takao, Hiroaki Kimura, Tetsuyuki Kitamoto, Ban Mihara
RNA binding proteins co-localize with small tau inclusions in tauopathy
Acta Neuropathologica Communications - Tập 6 - Trang 1-14 - 2018
Brandon F. Maziuk, Daniel J. Apicco, Anna Lourdes Cruz, Lulu Jiang, Peter E. A. Ash, Edroaldo Lummertz da Rocha, Cheng Zhang, Wai Haung Yu, John Leszyk, Jose F. Abisambra, Hu Li, Benjamin Wolozin
The development of insoluble, intracellular neurofibrillary tangles composed of the microtubule-associated protein tau is a defining feature of tauopathies, including Alzheimer’s disease (AD). Accumulating evidence suggests that tau pathology co-localizes with RNA binding proteins (RBPs) that are known markers for stress granules (SGs). Here we used proteomics to determine how the network of tau b...... hiện toàn bộ
Revisiting rodent models: Octodon degus as Alzheimer’s disease model?
Acta Neuropathologica Communications - Tập 4 - Trang 1-11 - 2016
Johannes Steffen, Markus Krohn, Kristin Paarmann, Christina Schwitlick, Thomas Brüning, Rita Marreiros, Andreas Müller-Schiffmann, Carsten Korth, Katharina Braun, Jens Pahnke
Alzheimer’s disease primarily occurs as sporadic disease and is accompanied with vast socio-economic problems. The mandatory basic research relies on robust and reliable disease models to overcome increasing incidence and emerging social challenges. Rodent models are most efficient, versatile, and predominantly used in research. However, only highly artificial and mostly genetically modified model...... hiện toàn bộ
Zidovudine ameliorates pathology in the mouse model of Duchenne muscular dystrophy via P2RX7 purinoceptor antagonism
Acta Neuropathologica Communications - Tập 6 - Trang 1-17 - 2018
Rasha Al-Khalidi, Chiara Panicucci, Paul Cox, Natalia Chira, Justyna Róg, Christopher N. J. Young, Rhiannon E. McGeehan, Kameshwari Ambati, Jayakrishna Ambati, Krzysztof Zabłocki, Elisabetta Gazzerro, Stephen Arkle, Claudio Bruno, Dariusz C. Górecki
Duchenne muscular dystrophy (DMD) is the most common inherited muscle disorder that causes severe disability and death of young men. This disease is characterized by progressive muscle degeneration aggravated by sterile inflammation and is also associated with cognitive impairment and low bone density. Given that no current treatment can improve the long-term outcome, approaches with a strong tran...... hiện toàn bộ
ALS-linked misfolded SOD1 species have divergent impacts on mitochondria
Acta Neuropathologica Communications - Tập 4 - Trang 1-14 - 2016
Sarah Pickles, Sabrina Semmler, Helen R. Broom, Laurie Destroismaisons, Laurine Legroux, Nathalie Arbour, Elizabeth Meiering, Neil R. Cashman, Christine Vande Velde
Approximately 20 % of familial Amyotrophic Lateral Sclerosis (ALS) is caused by mutations in superoxide dismutase (SOD1), which leads to misfolding of the SOD1 protein, resulting in a toxic gain of function. Several conformation-restricted antibodies have been generated that specifically recognize misfolded SOD1 protein, and have been used as therapeutics in pre-clinical models. Misfolded SOD1 sel...... hiện toàn bộ
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