A six degrees-of-freedom cable-driven robotic platform for head–neck movement by Ian Bales, Haohan Zhang

“…Poor postural control of the head–neck can be a debilitating symptom of neurological disorders such as amyotrophic lateral sclerosis and cerebral palsy. Current treatments using static neck collars are inadequate, and there is a need to develop new devices to empower movements and facilitate physical rehabilitation of the head–neck. …”
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Chronological requirements of TDP-43 function in synaptic organization and locomotive control by Giulia Romano, Raffaella Klima, Emanuele Buratti, Patrik Verstreken, Francisco E. Baralle, Fabian Feiguin

“…Alterations in TDP-43 are commonly found in patients suffering from amyotrophic lateral sclerosis (ALS) and the genetic suppression of the conserved homologue in Drosophila (TBPH) provokes alterations in the functional organization of motoneuron synaptic terminals, resulting in locomotive defects and reduced life span. …”
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Auditory attention measured by EEG in neurological populations: systematic review of literature and meta-analysis by Nele Vanbilsen, Sonja A. Kotz, Mattia Rosso, Marc Leman, Lisa Tedesco Triccas, Peter Feys, Lousin Moumdjian

“…In total, 35 studies were included, including participants with Parkinson’s disease (PD), stroke, Traumatic Brain Injury (TBI), Multiple Sclerosis (MS), Amyotrophic Lateral Sclerosis (ALS). A meta-analysis was performed on P3 amplitude and latency separately to look at the differences between neurological populations and healthy controls in terms of P3 amplitude and latency. …”
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Brain-Computer Interface-Based Communication in the Completely Locked-In State. by Ujwal Chaudhary, Bin Xia, Stefano Silvoni, Leonardo G Cohen, Niels Birbaumer

“…Four patients suffering from advanced amyotrophic lateral sclerosis (ALS)-two of them in permanent CLIS and two entering the CLIS without reliable means of communication-learned to answer personal questions with known answers and open questions all requiring a "yes" or "no" thought using frontocentral oxygenation changes measured with fNIRS. …”
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Structure of the human C9orf72-SMCR8 complex reveals a multivalent protein interaction architecture. by Julia Nörpel, Simone Cavadini, Andreas D Schenk, Alexandra Graff-Meyer, Daniel Hess, Jan Seebacher, Jeffrey A Chao, Varun Bhaskar

“…A major cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) spectrum disorder is the hexanucleotide G4C2 repeat expansion in the first intron of the C9orf72 gene. …”
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Loss of function CHCHD10 mutations in cytoplasmic TDP-43 accumulation and synaptic integrity by Jung-A. A. Woo, Tian Liu, Courtney Trotter, Cenxiao C. Fang, Emillio De Narvaez, Patrick LePochat, Drew Maslar, Anusha Bukhari, Xingyu Zhao, Andrew Deonarine, Sandy D. Westerheide, David E. Kang

“…Mutations inCHCHD10 have been recently associated with frontotemporal dementia and amyotrophic lateral sclerosis. Here the authors study the functions of endogenous CHCHD10 in Caenorhabditis elegans, primary neurons, and mouse, and show that it normally protects mitochondria and synaptic integrity, and retains TDP-43 in the nucleus.…”
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Plasma neurofilament heavy chain levels correlate to markers of late stage disease progression and treatment response in SOD1(G93A) mice that model ALS. by Ching-Hua Lu, Axel Petzold, Bernadett Kalmar, James Dick, Andrea Malaspina, Linda Greensmith

“…Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disorder characterised by progressive degeneration of motor neurons leading to death, typically within 3-5 years of symptom onset. …”
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The role of macrophage plasticity in neurodegenerative diseases by Hongyue Ma, Mingxia Zhu, Mengjie Chen, Xiuli Li, Xinhong Feng

“…Dysregulated states of macrophages can compromise clearance mechanisms for pathological proteins such as amyloid-β (Aβ) in Alzheimer’s disease (AD) and TDP-43 in Amyotrophic lateral sclerosis (ALS). Additionally, recent evidence suggests that abnormalities in the peripheral clearance of pathological proteins are implicated in the pathogenesis and progression of neurodegenerative diseases. …”
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Riluzole as a Neuroprotective Drug for Spinal Cord Injury: From Bench to Bedside by Narihito Nagoshi, Hiroaki Nakashima, Michael G. Fehlings

“…Food and Drug Administration as a safe and well-tolerated treatment for patients with amyotrophic lateral sclerosis. The mechanism of action of riluzole involves the inhibition of pathologic glutamatergic transmission in synapses of neurons via sodium channel blockade. …”
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Drosophila lines with mutant and wild type human TDP-43 replacing the endogenous gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or life... by Jer-Cherng Chang, David B Morton

“…Mutations in TDP-43 are associated with proteinaceous inclusions in neurons and are believed to be causative in neurodegenerative diseases such as frontotemporal dementia or amyotrophic lateral sclerosis. Here we describe a Drosophila system where we have engineered the genome to replace the endogenous TDP-43 orthologue with wild type or mutant human TDP-43(hTDP-43). …”
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Knockdown of the Drosophila fused in sarcoma (FUS) homologue causes deficient locomotive behavior and shortening of motoneuron terminal branches. by Hiroshi Sasayama, Mai Shimamura, Takahiko Tokuda, Yumiko Azuma, Tomokatsu Yoshida, Toshiki Mizuno, Masanori Nakagawa, Nobuhiro Fujikake, Yoshitaka Nagai, Masamitsu Yamaguchi

“…Mutations in the fused in sarcoma/translated in liposarcoma gene (FUS/TLS, FUS) have been identified in sporadic and familial forms of amyotrophic lateral sclerosis (ALS). FUS is an RNA-binding protein that is normally localized in the nucleus, but is mislocalized to the cytoplasm in ALS, and comprises cytoplasmic inclusions in ALS-affected areas. …”
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Functional implication of ubiquitinating and deubiquitinating mechanisms in TDP-43 proteinopathies by Non-Nuoc Tran, Byung-Hoon Lee, Byung-Hoon Lee

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease in which motor neurons in spinal cord and motor cortex are progressively lost. …”
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Rab5 and Alsin regulate stress-activated cytoprotective signaling on mitochondria by FoSheng Hsu, Stephanie Spannl, Charles Ferguson, Anthony A Hyman, Robert G Parton, Marino Zerial

“…Interestingly, activation of Rab5 on mitochondria depends on the Rab5-GEF ALS2/Alsin, encoded by a gene mutated in amyotrophic lateral sclerosis (ALS). Alsin-deficient human-induced pluripotent stem cell-derived spinal motor neurons are defective in relocating Rab5 to mitochondria and display increased susceptibility to oxidative stress. …”
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Tracking mesenchymal stem cells using magnetic resonance imaging by Jens T Rosenberg, Xuegang Yuan, Samuel Grant, Teng Ma

“…Recent translational studies in the fields of tissue regeneration and cell therapy have characterized mesenchymal stem cells (MSCs) as a potentially effective and accessible measure for treating ischemic cerebral and neurodegenerative disorders such as stroke, Parkinson's disease, and amyotrophic lateral sclerosis. Developing more efficient cell tracking techniques bear the potential to optimize MSC transplantation therapies by providing a more accurate picture of the fate and area of effect of implanted cells. …”
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Protective role of glutathione in oxidative stress caused by cadmium and copper by J. Jovanovic Mirkovic, G. Kocic, Z. Jurinjak, C. Alexopoulos

“…Oxidative stress occurs due to increased production of reactive oxygen species (Parkinson’s and Alzheimer’s disease) or reduced ability of cells to neutralize it through their internal antioxidants (eg mutation of the superoxide dismutase gene in amyotrophic lateral sclerosis). Objectives The aim of this research was to examine the protective role of supplement, GSH, S-donor ligand, and in conditions of acute and chronic intoxication with sublethal doses of cadmium-II-chloride and copper II sulfate. …”
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The Big Picture of Neurodegeneration: A Meta Study to Extract the Essential Evidence on Neurodegenerative Diseases in a Network-Based Approach by Nicolas Ruffini, Nicolas Ruffini, Susanne Klingenberg, Raoul Heese, Susann Schweiger, Susanne Gerber

“…The common features of all neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, Amyotrophic Lateral Sclerosis (ALS), and Huntington's disease, are the accumulation of aggregated and misfolded proteins and the progressive loss of neurons, leading to cognitive decline and locomotive dysfunction. …”
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The interplay between iron accumulation, mitochondrial dysfunction and inflammation during the execution step of neurodegenerative disorders by Pamela J. Urrutia, Natalia P. Mena, Marco Tulio Nunez

“…A growing set of observations points to mitochondrial dysfunction, iron accumulation, oxidative damage and chronic inflammation as common pathognomonic signs of a number of neurodegenerative diseases that includes Alzheimer's disease, Huntington disease, amyotrophic lateral sclerosis, Friedrich’s ataxia and Parkinson’s disease. …”
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Advanced Glycation End Products in Health and Disease by V. Prakash Reddy, Puspa Aryal, Emmanuel K. Darkwah

“…The overexpression of RAGE and the AGE-RAGE interactions are especially involved in cases of Alzheimer’s disease and other neurodegenerative diseases, including TBI and amyotrophic lateral sclerosis (ALS). Maillard reactions are also observed in the gut bacterial species. …”
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Proteostasis Disturbances and Inflammation in Neurodegenerative Diseases by Tuuli-Maria Sonninen, Gundars Goldsteins, Nihay Laham-Karam, Jari Koistinaho, Šárka Lehtonen

“…Here, we review the mechanisms of proteostasis and inflammatory response, emphasizing their role in the pathological hallmarks of neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis. Furthermore, we discuss the interplay between proteostatic stress and excessive immune response that activates inflammation and leads to dysfunctional proteostasis.…”
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The cost-effectiveness of early noninvasive ventilation for ALS patients by Chernew Michael E, Gruis Kirsten L, Brown Devin L

“…<p>Abstract</p> <p>Background</p> <p>Optimal timing of noninvasive positive pressure ventilation (NIPPV) initiation in patients with amyotrophic lateral sclerosis (ALS) is unknown, but NIPPV appears to benefit ALS patients who are symptomatic from pulmonary insufficiency. …”
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