Results 41 to 50 of about 2,351,931 (407)

Unbiased Label-Free Quantitative Proteomics of Cells Expressing Amyotrophic Lateral Sclerosis (ALS) Mutations in CCNF Reveals Activation of the Apoptosis Pathway: A Workflow to Screen Pathogenic Gene Mutations

Frontiers in Molecular Neuroscience, 2021
The past decade has seen a rapid acceleration in the discovery of new genetic causes of ALS, with more than 20 putative ALS-causing genes now cited. These genes encode proteins that cover a diverse range of molecular functions, including free radical ...
Flora Cheng, Alana De Luca, Alison L. Hogan, Stephanie L. Rayner, Jennilee M. Davidson, Maxinne Watchon, Claire H. Stevens, Claire H. Stevens, Sonia Sanz Muñoz, Sonia Sanz Muñoz, Lezanne Ooi, Lezanne Ooi, Justin J. Yerbury, Justin J. Yerbury, Emily K. Don, Jennifer A. Fifita, Maria D. Villalva, Hannah Suddull, Tyler R. Chapman, Thomas J. Hedl, Adam K. Walker, Adam K. Walker, Shu Yang, Marco Morsch, Bingyang Shi, Ian P. Blair, Angela S. Laird, Roger S. Chung, Albert Lee   +28 more
doaj   +1 more source

Reduced sensory synaptic excitation impairs motor neuron function via Kv2.1 in spinal muscular atrophy

Nature Neuroscience, 2017
Behavioral deficits in neurodegenerative diseases are often attributed to the selective dysfunction of vulnerable neurons via cell-autonomous mechanisms.
Emily V. Fletcher, C. Simon, John G. Pagiazitis, Joshua I. Chalif, Aleksandra Vukojicic, Estelle Drobac, Xiaojian Wang, G. Mentis   +7 more
semanticscholar   +1 more source

Unsolicited written narratives as a methodological genre in terminal illness: challenges and limitations [PDF]

, 2011
Stories about illness have proven invaluable in helping health professionals understand illness experiences. Such narratives have traditionally been solicited by researchers through interviews and the collection of personal writings, including diaries ...
Bazeley P., David Clark, Grinyer A., Hawkins A. H., Mary R. O’Brien, Miles M. B., Motor Neuron Disease Association   +6 more
core   +1 more source

Modeling Environmentally-Induced Motor Neuron Degeneration in Zebrafish

Scientific Reports, 2018
Zebrafish have been used to investigate motor neuron degeneration, including as a model system to examine the pathogenesis of amyotrophic lateral sclerosis (ALS). The use of zebrafish for this purpose has some advantages over other in vivo model systems.
J. R. Morrice, C. Gregory‐Evans, C. A. Shaw   +2 more
semanticscholar   +1 more source

Motor Neurons that Multitask [PDF]

Neuron, 2012
Animals use a form of sensory feedback termed proprioception to monitor their body position and modify the motor programs that control movement. In this issue of Neuron, Wen et al. (2012) provide evidence that a subset of motor neurons function as proprioceptors in C.
openaire   +3 more sources

The converging roles of sequestosome-1/p62 in the molecular pathways of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)

Neurobiology of Disease, 2022
Investigations into the pathogenetic mechanisms underlying amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have provided significant insight into the disease.
Jennilee M. Davidson, Roger S. Chung, Albert Lee   +2 more
doaj   +1 more source

Direct conversion of human pluripotent stem cells into cranial motor neurons using a piggyBac vector [PDF]

, 2018
Human pluripotent stem cells (PSCs) are widely used for in vitro disease modeling. One of the challenges in the field is represented by the ability of converting human PSCs into specific disease-relevant cell types.
De Santis, Riccardo, de Turris, Valeria, Di Angelantonio, Silvia, Garone, Maria Giovanna, Pagani, Francesca, Rosa, Alessandro   +5 more
core   +2 more sources

Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis

Acta Neuropathologica, 2017
Motor neuron-extrinsic mechanisms have been shown to participate in the pathogenesis of ALS-SOD1, one familial form of amyotrophic lateral sclerosis (ALS).
Jelena Scekic-Zahirovic, Hajer El Oussini, Sina Mersmann, Kevin Drenner, Marina Wagner, Ying Sun, K. Allmeroth, S. Dieterlé, J. Sinniger, S. Dirrig‐Grosch, F. René, D. Dormann, C. Haass, A. Ludolph, C. Lagier-Tourenne, E. Storkebaum, L. Dupuis   +16 more
semanticscholar   +1 more source

State Variables of the Arm May Be Encoded by Single Neuron Activity in the Monkey Motor Cortex [PDF]

, 2016
Revealing the type of information encoded by neurons activity in the motor cortex is essential not only for understanding the mechanism of motion control but also for developing a brain-machine interface.
Eizo Miyashita, Yutaka Sakaguchi
core   +2 more sources

Real-time visualization of oxidative stress-mediated neurodegeneration of individual spinal motor neurons in vivo

Redox Biology, 2018
Generation of reactive oxygen species (ROS) has been shown to be important for many physiological processes, ranging from cell differentiation to apoptosis. With the development of the genetically encoded photosensitiser KillerRed (KR) it is now possible
Isabel Formella, Adam J. Svahn, Rowan A.W. Radford, Emily K. Don, Nicholas J. Cole, Alison Hogan, Albert Lee, Roger S. Chung, Marco Morsch   +8 more
doaj   +1 more source