Results 11 to 20 of about 390,159 (364)

Motor Neuron Diseases

DeckerMed Neurology, 2022
The motor neuron diseases (MNDs) are a family of diseases commonly categorized by their propensity to affect upper or lower motor neurons and by their mode of inheritance. The chapter provides some content on infectious MNDs caused by viral infections affecting the motor neurons in the anterior horn of the spinal cord. However, the chapter devotes most
Elena Ratti, Merit Cudkowicz, James D. Berry   +2 more
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Defective axonal transport in motor neuron disease [PDF]

, 2007
Several recent studies have highlighted the role of axonal transport in the pathogenesis of motor neuron diseases. Mutations in genes that control microtubule regulation and dynamics have been shown to cause motor neuron degeneration in mice and in a ...
Baas, Blair, Boillee, Bommel, Borchelt, Bowling, Brown, Bruijn, Burger, Cao, Cleveland, Cleveland, Duchen, Escurat, Evans, Fichera, Fliegner, Gaudette, Goldstein, Hadano, Hafezparast, Hazan, Hirokawa, Hirokawa, Hirokawa, Jablonka, Joshi, Kanai, Kaplan, Kieran, King, Kong, LaMonte, Levy, Ligon, Liu, Lo Giudice, Lu, Martin, Miki, Miki, Mitsumoto, Mitsumoto, Muglia, Munch, Nicholson, Nishimura, Okabe, Otomo, Parkinson, Parysek, Pasinelli, Patel, Proukakis, Puls, Puls, Quenneville, Rao, Reid, Reid, Reid, Rosen, Ross, Rowland, Sanderson, Schmitt-John, Shaw, Shibata, Siegel, Song, Teuchert, Troy, Vallee, Weber, Williamson, Wood, Xia, Zhao   +77 more
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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
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Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy

Cell Reports, 2017
The hallmark of spinal muscular atrophy (SMA), an inherited disease caused by ubiquitous deficiency in the SMN protein, is the selective degeneration of subsets of spinal motor neurons.
Christian M. Simon, Ya Dai, Meaghan Van Alstyne, Charalampia Koutsioumpa, John G. Pagiazitis, Joshua I. Chalif, Xiaojian Wang, Joseph E. Rabinowitz, Christopher E. Henderson, Livio Pellizzoni, George Z. Mentis   +10 more
doaj   +1 more source

Neurons other than motor neurons in motor neuron disease.

Histology and histopathology, 2017
Amyotrophic lateral sclerosis (ALS) is typically defined by a loss of motor neurons in the central nervous system. Accordingly, morphological analysis for decades considered motor neurons (in the cortex, brainstem and spinal cord) as the neuronal population selectively involved in ALS.
Ruffoli R, Biagioni F, Busceti CL, Gaglione A, Ryskalin L, Gambardella S, Frati A, Fornai F.   +7 more
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The era of cryptic exons: implications for ALS-FTD

Molecular Neurodegeneration, 2023
TDP-43 is an RNA-binding protein with a crucial nuclear role in splicing, and mislocalises from the nucleus to the cytoplasm in a range of neurodegenerative disorders.
Puja R. Mehta, Anna-Leigh Brown, Michael E. Ward, Pietro Fratta   +3 more
doaj   +1 more source

Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease [PDF]

, 2018
Human Ag R (HuR) is an RNA binding protein in the ELAVL protein family. To study the neuron-specific function of HuR, we generated inducible, neuron-specific HuR-deficient mice of both sexes.
Bai, Ying, Chen, Xing, Hamilton, Thomas A., Herjan, Tomasz, Kokiko-Cochran, Olga N., Lamb, Bruce, Lee, Yu-Shang, Li, Xiao, Lin, Ching-Yi, Sun, Kevin, Zhou, Gao   +10 more
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Motor neurons and the generation of spinal motor neuron diversity [PDF]

Frontiers in Cellular Neuroscience, 2014
Motor neurons (MNs) are neuronal cells located in the central nervous system (CNS) controlling a variety of downstream targets. This function infers the existence of MN subtypes matching the identity of the targets they innervate. To illustrate the mechanism involved in the generation of cellular diversity and the acquisition of specific identity, this
openaire   +4 more sources

A Novel Calpain Inhibitor Compound Has Protective Effects on a Zebrafish Model of Spinocerebellar Ataxia Type 3

Cells, 2021
Spinocerebellar ataxia type 3 (SCA3) is a hereditary ataxia caused by inheritance of a mutated form of the human ATXN3 gene containing an expanded CAG repeat region, encoding a human ataxin-3 protein with a long polyglutamine (polyQ) repeat region ...
Katherine J. Robinson, Kristy Yuan, Stuart K. Plenderleith, Maxinne Watchon, Angela S. Laird   +4 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
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