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Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions
Published 2018-01-01“…TAR DNA-binding protein-43 KDa (TDP-43) and fused in sarcoma (FUS) as the defining pathological hallmarks for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), coupled with ALS-FTD-causing mutations in both genes, indicate that their dysfunctions damage the motor system and cognition. …”
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62
Toll-Like Receptors Expression and Signaling in Glia Cells in Neuro-Amyloidogenic Diseases: Towards Future Therapeutic Application
Published 2010-01-01“…This paper will focus on TLR signaling in glial cells in neurodegenerative diseases such as Alzheimer's disease, prion diseases, amyotrophic lateral sclerosis, and Parkinson's disease. Understanding the pattern of TLR signaling in the glial cells may lead to the identification of new targets for therapeutic application.…”
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63
ER Dysfunction and Protein Folding Stress in ALS
Published 2013-01-01“…Amyotrophic lateral sclerosis (ALS) is the most frequent paralytic disease in adults. …”
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64
Communication Support for People with ALS
Published 2011-01-01“…Almost all people with amyotrophic lateral sclerosis (ALS) experience a motor speech disorder, such as dysarthria, as the disease progresses. …”
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65
Biophysical and spectroscopical insights into structural modulation of species in the aggregation pathway of superoxide dismutase 1
Published 2025-01-01“…Abstract Superoxide dismutase 1 (SOD1) aggregation is implicated in the development of Amyotrophic Lateral Sclerosis (ALS). Despite knowledge of the role of SOD1 aggregation, the mechanistic understanding remains elusive. …”
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66
Friends or Foes: Matrix Metalloproteinases and Their Multifaceted Roles in Neurodegenerative Diseases
Published 2015-01-01“…Here, we review MMPs and their roles in neurodegenerative diseases: Alzheimer’s disease (AD), Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), Huntington’s disease (HD), and multiple sclerosis (MS). …”
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67
Generating Diverse Spinal Motor Neuron Subtypes from Human Pluripotent Stem Cells
Published 2016-01-01“…Motor neurons (MNs) represent a diverse pool of neuronal subtypes exhibiting differential vulnerability in different human neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). …”
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68
Advanced Skeletal Muscle Mass Reduction (Sarcopenia) Secondary to Neuromuscular Disease
Published 2020-01-01“…We describe a young male patient chronically on a ventilator secondary to decreased mobility from amyotrophic lateral sclerosis (ALS). He had both a tracheostomy for breathing and percutaneous endoscopic gastrostomy (PEG) for feeding. …”
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69
Regulation of Glial Cell Functions by PPAR-𝜸 Natural and Synthetic Agonists
Published 2008-01-01“…PPAR-𝛾 agonists, which include naturally occurring compounds (such as long chain fatty acids and the cyclopentenone prostaglandin 15-deoxy Δ12,14 prostaglandin J2), and synthetic agonists (among which the thiazolidinediones and few nonsteroidal anti-inflammatory drugs) have shown anti-inflammatory and protective effects in several experimental models of Alzheimer's and Parkinson's diseases, amyotrophic lateral sclerosis, multiple sclerosis and stroke, as well as in few clinical studies. …”
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70
D-Amino Acids in the Nervous and Endocrine Systems
Published 2016-01-01“…Aberrant receptor activity is related to diseases of the central nervous system (CNS), such as Alzheimer’s disease, amyotrophic lateral sclerosis, and schizophrenia. Furthermore, D-amino acids are detected in parts of the endocrine system, such as the pineal gland, hypothalamus, pituitary gland, pancreas, adrenal gland, and testis. …”
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71
Alternative 3′ UTR polyadenylation is disrupted in the rNLS8 mouse model of ALS/FTLD
Published 2025-01-01“…Abstract Recent research has highlighted widespread dysregulation of alternative polyadenylation in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP). …”
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72
What is “Hyper” in the ALS Hypermetabolism?
Published 2017-01-01“…The progressive and fatal loss of upper (brain) and lower (spinal cord) motor neurons and muscle denervation concisely condenses the clinical picture of amyotrophic lateral sclerosis (ALS). Despite the multiple mechanisms believed to underlie the selective loss of motor neurons, ALS aetiology remains elusive and obscure. …”
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73
Therapeutic Use of Botulinum Toxin in Neurorehabilitation
Published 2012-01-01“…The use of BTX for the rehabilitation of children with obstetrical brachial plexus palsy and in treating sialorrhea which can complicate the course of some severe neurological diseases such as amyotrophic lateral sclerosis and Parkinson's disease is also addressed. …”
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74
Neuroinflammation and ALS: Transcriptomic Insights into Molecular Disease Mechanisms and Therapeutic Targets
Published 2017-01-01“…Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the motor nervous system. …”
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75
SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
Published 2011-01-01“…The discovery that mutations in SOD1 gene cause a subset of familial amyotrophic lateral sclerosis (FALS) has attracted great attention, and studies to date have been mainly focused on discovering mutations in the coding region and investigation at protein level. …”
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76
Cognitive Impairment Involving Social Cognition in SPG4 Hereditary Spastic Paraplegia
Published 2016-01-01“…SPG4 phenotypes share some cognitive features of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Cognitive disorders including executive disorders and social cognition impairment are frequent in SPG4 patients and might sometimes occur before motor disorders. …”
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77
Hsp70 and Its Molecular Role in Nervous System Diseases
Published 2011-01-01“…Hsp70 functions as a chaperone and protects neurons from protein aggregation and toxicity (Parkinson disease, Alzheimer disease, polyglutamine diseases, and amyotrophic lateral sclerosis), protects cells from apoptosis (Parkinson disease), is a stress marker (temporal lobe epilepsy), protects cells from inflammation (cerebral ischemic injury), has an adjuvant role in antigen presentation and is involved in the immune response in autoimmune disease (multiple sclerosis). …”
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78
A proof-of-concept study of extracting patient histories for rare/intractable diseases from social media
Published 2020-06-01“…We selected amyotrophic lateral sclerosis and multiple sclerosis as use cases of rare and intractable diseases, respectively, and we extracted patient histories related to these health conditions from Twitter. …”
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79
Involvement of Coenzyme Q10 in Various Neurodegenerative and Psychiatric Diseases
Published 2023-01-01“…This agent has shown its efficacy in preventing and treating neurological diseases such as migraine, Parkinson’s disease, Alzheimer’s disease, Huntington’s disease, amyotrophic lateral sclerosis, and Friedreich’s ataxia. This study reviews the literature to highlight this agent’s potential therapeutic effects in the mentioned neurological disorders.…”
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80
Gamisoyo-San Ameliorates Neuroinflammation in the Spinal Cord of hSOD1G93A Transgenic Mice
Published 2018-01-01“…Amyotrophic lateral sclerosis (ALS), a progressive disorder, causes motor neuron degeneration and neuromuscular synapse denervation. …”
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