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Artículos de revistas sobre el tema "Molecular aspects of Amyotrophic lateral sclerosis"

Autor: Grafiati

Publicado: 4 de junio de 2021

Última modificación: 28 de julio de 2025

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1

Dilliott, Allison A., Catherine M. Andary, Meaghan Stoltz, Andrey A. Petropavlovskiy, Sali M. K. Farhan, and Martin L. Duennwald. "DnaJC7 in Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 23, no. 8 (2022): 4076. http://dx.doi.org/10.3390/ijms23084076.

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Protein misfolding is a common basis of many neurodegenerative diseases including amyotrophic lateral sclerosis (ALS). Misfolded proteins, such as TDP-43, FUS, Matrin3, and SOD1, mislocalize and form the hallmark cytoplasmic and nuclear inclusions in neurons of ALS patients. Cellular protein quality control prevents protein misfolding under normal conditions and, particularly, when cells experience protein folding stress due to the fact of increased levels of reactive oxygen species, genetic mutations, or aging. Molecular chaperones can prevent protein misfolding, refold misfolded proteins, or

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2

Strong, Michael J., and Ralph M. Garruto. "Chronic Aluminum-Induced Motor Neuron Degeneration: Clinical, Neuropathological and Molecular Biological Aspects." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 18, S3 (1991): 428–31. http://dx.doi.org/10.1017/s0317167100032601.

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ABSTRACT:The monthly intracisternal inoculation of young adult New Zealand white rabbits with low-dose (100 Μg) aluminum chloride induces aggregates of phosphorylated neurofilament that mimics the intraneuronal inclusions of amyotrophic lateral sclerosis. The chronic progressive myelopathy and topographically-specific motor neuron degeneration that occurs in the absence of suppressions of neurofilament messenger RNA levels in this model contrasts with the acute fulminant encephalomyelopathy and nonspecific gene suppressions that occur subsequent to high-dose (1000 Μg) aluminum chloride inocula

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3

Grunseich, Christopher, Aneesh Patankar, Joshua Amaya, et al. "Clinical and Molecular Aspects of Senataxin Mutations in Amyotrophic Lateral Sclerosis 4." Annals of Neurology 87, no. 4 (2020): 547–55. http://dx.doi.org/10.1002/ana.25681.

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4

Jankovic, Milena, Ivana Novakovic, Phepy Gamil Anwar Dawod, et al. "Current Concepts on Genetic Aspects of Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 22, no. 18 (2021): 9832. http://dx.doi.org/10.3390/ijms22189832.

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Amyotrophic Lateral Sclerosis (ALS), neurodegenerative motor neuron disorder is characterized as multisystem disease with important contribution of genetic factors. The etiopahogenesis of ALS is not fully elucidate, but the dominant theory at present relates to RNA processing, as well as protein aggregation and miss-folding, oxidative stress, glutamate excitotoxicity, inflammation and epigenetic dysregulation. Additionally, as mitochondria plays a leading role in cellular homeostasis maintenance, a rising amount of evidence indicates mitochondrial dysfunction as a substantial contributor to di

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5

Bampton, Alexander, Lauren M. Gittings, Pietro Fratta, Tammaryn Lashley, and Ariana Gatt. "The role of hnRNPs in frontotemporal dementia and amyotrophic lateral sclerosis." Acta Neuropathologica 140, no. 5 (2020): 599–623. http://dx.doi.org/10.1007/s00401-020-02203-0.

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Abstract Dysregulated RNA metabolism is emerging as a crucially important mechanism underpinning the pathogenesis of frontotemporal dementia (FTD) and the clinically, genetically and pathologically overlapping disorder of amyotrophic lateral sclerosis (ALS). Heterogeneous nuclear ribonucleoproteins (hnRNPs) comprise a family of RNA-binding proteins with diverse, multi-functional roles across all aspects of mRNA processing. The role of these proteins in neurodegeneration is far from understood. Here, we review some of the unifying mechanisms by which hnRNPs have been directly or indirectly link

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6

Stangl, Michaela, Wolfgang Böcker, Vladimir Chubanov, et al. "Sarcopenia – Endocrinological and Neurological Aspects." Experimental and Clinical Endocrinology & Diabetes 6, no. 01 (2018): 8–22. http://dx.doi.org/10.1055/a-0672-1007.

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AbstractSarcopenia in geriatric patients is often associated with or even caused by changes of the endocrine and nervous system. The multifactorial pathogenesis of sarcopenia and additional multimorbidity in geriatric patients makes it difficult to study distinct pathogenic pathways leading to sarcopenia. Patients suffering from diabetes, Cushing’s syndrome, chronic kidney disease, Klinefelter’s syndrome or motor neuron diseases, such as amyotrophic lateral sclerosis for example are known to have impaired muscle property and reduced physical performance. These patients are typically younger an

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7

De Giorgio, Francesca, Cheryl Maduro, Elizabeth M. C. Fisher, and Abraham Acevedo-Arozena. "Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis." Disease Models & Mechanisms 12, no. 1 (2019): dmm037424. http://dx.doi.org/10.1242/dmm.037424.

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8

Cho, HaEun, and Surabhi Shukla. "Role of Edaravone as a Treatment Option for Patients with Amyotrophic Lateral Sclerosis." Pharmaceuticals 14, no. 1 (2020): 29. http://dx.doi.org/10.3390/ph14010029.

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Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive and fatal neurodegenerative disease that leads to a loss of muscle control due to nerve cells being affected in the brain and spinal cord. Some of the common clinical presentations of ALS include weakness of muscles, changes in behavior, dysfunction in speech, and cognitive difficulties. The cause of ALS is uncertain, but through several studies, it is known that mutations in SOD1 or C9orf72 genes could play a role as a factor of ALS. In addition, studies indicate that an excessive amount of free radicals

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9

Tzeplaeff, Laura, Alexandra V. Jürs, Camilla Wohnrade, and Antonia F. Demleitner. "Unraveling the Heterogeneity of ALS—A Call to Redefine Patient Stratification for Better Outcomes in Clinical Trials." Cells 13, no. 5 (2024): 452. http://dx.doi.org/10.3390/cells13050452.

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Despite tremendous efforts in basic research and a growing number of clinical trials aiming to find effective treatments, amyotrophic lateral sclerosis (ALS) remains an incurable disease. One possible reason for the lack of effective causative treatment options is that ALS may not be a single disease entity but rather may represent a clinical syndrome, with diverse genetic and molecular causes, histopathological alterations, and subsequent clinical presentations contributing to its complexity and variability among individuals. Defining a way to subcluster ALS patients is becoming a central end

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10

Wright, Gareth S. A. "Molecular and pharmacological chaperones for SOD1." Biochemical Society Transactions 48, no. 4 (2020): 1795–806. http://dx.doi.org/10.1042/bst20200318.

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The efficacy of superoxide dismutase-1 (SOD1) folding impacts neuronal loss in motor system neurodegenerative diseases. Mutations can prevent SOD1 post-translational processing leading to misfolding and cytoplasmic aggregation in familial amyotrophic lateral sclerosis (ALS). Evidence of immature, wild-type SOD1 misfolding has also been observed in sporadic ALS, non-SOD1 familial ALS and Parkinson's disease. The copper chaperone for SOD1 (hCCS) is a dedicated and specific chaperone that assists SOD1 folding and maturation to produce the active enzyme. Misfolded or misfolding prone SOD1 also int

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11

Lesman-Segev, Orit H., Lauren Edwards, and Gil D. Rabinovici. "Chronic Traumatic Encephalopathy: A Comparison with Alzheimer's Disease and Frontotemporal Dementia." Seminars in Neurology 40, no. 04 (2020): 394–410. http://dx.doi.org/10.1055/s-0040-1715134.

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AbstractThe clinical diagnosis of chronic traumatic encephalopathy (CTE) is challenging due to heterogeneous clinical presentations and overlap with other neurodegenerative dementias. Depending on the clinical presentation, the differential diagnosis of CTE includes Alzheimer's disease (AD), behavioral variant frontotemporal dementia (bvFTD), Parkinson's disease, amyotrophic lateral sclerosis, primary mood disorders, posttraumatic stress disorder, and psychotic disorders. The aim of this article is to compare the clinical aspects, genetics, fluid biomarkers, imaging, treatment, and pathology o

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12

Genc, Baris, Oge Gozutok, and P. Hande Ozdinler. "Complexity of Generating Mouse Models to Study the Upper Motor Neurons: Let Us Shift Focus from Mice to Neurons." International Journal of Molecular Sciences 20, no. 16 (2019): 3848. http://dx.doi.org/10.3390/ijms20163848.

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Motor neuron circuitry is one of the most elaborate circuitries in our body, which ensures voluntary and skilled movement that requires cognitive input. Therefore, both the cortex and the spinal cord are involved. The cortex has special importance for motor neuron diseases, in which initiation and modulation of voluntary movement is affected. Amyotrophic lateral sclerosis (ALS) is defined by the progressive degeneration of both the upper and lower motor neurons, whereas hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS) are characterized mainly by the loss of upper motor n

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13

Schweingruber, Christoph, and Eva Hedlund. "The Cell Autonomous and Non-Cell Autonomous Aspects of Neuronal Vulnerability and Resilience in Amyotrophic Lateral Sclerosis." Biology 11, no. 8 (2022): 1191. http://dx.doi.org/10.3390/biology11081191.

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Amyotrophic lateral sclerosis (ALS) is defined by the loss of upper motor neurons (MNs) that project from the cerebral cortex to the brain stem and spinal cord and of lower MNs in the brain stem and spinal cord which innervate skeletal muscles, leading to spasticity, muscle atrophy, and paralysis. ALS involves several disease stages, and multiple cell types show dysfunction and play important roles during distinct phases of disease initiation and progression, subsequently leading to selective MN loss. Why MNs are particularly vulnerable in this lethal disease is still not entirely clear. Neith

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14

Retinasamy, Thaarvena, and Mohd Farooq Shaikh. "Interleukin 1 receptor antagonist and neurodegenerative diseases: the future treatment strategy." Neuroscience Research Notes 6, no. 1 (2023): 164. http://dx.doi.org/10.31117/neuroscirn.v6i1.164.

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Neurodegenerative disorders encompass a range of conditions affecting the central nervous system (CNS) in which alterations in the neuronal structure and cellular dysfunction lead to progressive deterioration. Activation of microglia and expression of the inflammatory cytokine interleukin-1 (IL-1) in the CNS have become almost synonymous with neuroinflammation. Additionally, the relentless activation of the IL-1 signalling pathway has been linked with the pathogenesis of various CNS disease states, ranging from Alzheimer disease (AD), Parkinson disease (PD) to amyotrophic lateral sclerosis (AL

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15

Angeloni, Cristina, Martina Gatti, Cecilia Prata, Silvana Hrelia, and Tullia Maraldi. "Role of Mesenchymal Stem Cells in Counteracting Oxidative Stress—Related Neurodegeneration." International Journal of Molecular Sciences 21, no. 9 (2020): 3299. http://dx.doi.org/10.3390/ijms21093299.

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Neurodegenerative diseases include a variety of pathologies such as Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, amyotrophic lateral sclerosis, and so forth, which share many common characteristics such as oxidative stress, glycation, abnormal protein deposition, inflammation, and progressive neuronal loss. The last century has witnessed significant research to identify mechanisms and risk factors contributing to the complex etiopathogenesis of neurodegenerative diseases, such as genetic, vascular/metabolic, and lifestyle-related factors, which often co-occur and interact wi

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16

Rossaert, Elisabeth, Eveliina Pollari, Tom Jaspers, et al. "Restoration of histone acetylation ameliorates disease and metabolic abnormalities in a FUS mouse model." Acta Neuropathologica Communications 7, no. 1 (2019): 107. https://doi.org/10.1186/s40478-019-0750-2.

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Dysregulation of epigenetic mechanisms is emerging as a central event in neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS). In many models of neurodegeneration, global histone acetylation is decreased in the affected neuronal tissues. Histone acetylation is controlled by the antagonistic actions of two protein families –the histone acetyltransferases (HATs) and the histone deacetylases (HDACs). Drugs inhibiting HDAC activity are already used in the clinic as anti-cancer agents. The aim of this study was to explore the therapeutic potential of HDAC inhibition in the con

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17

Galvez-Llompart, Maria, Riccardo Zanni, Ramon Garcia-Domenech, and Jorge Galvez. "How Molecular Topology Can Help in Amyotrophic Lateral Sclerosis (ALS) Drug Development: A Revolutionary Paradigm for a Merciless Disease." Pharmaceuticals 15, no. 1 (2022): 94. http://dx.doi.org/10.3390/ph15010094.

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Even if amyotrophic lateral sclerosis is still considered an orphan disease to date, its prevalence among the population is growing fast. Despite the efforts made by researchers and pharmaceutical companies, the cryptic information related to the biological and physiological onset mechanisms, as well as the complexity in identifying specific pharmacological targets, make it almost impossible to find effective treatments. Furthermore, because of complex ethical and economic aspects, it is usually hard to find all the necessary resources when searching for drugs for new orphan diseases. In this

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18

Margiotta, Azzurra. "Role of SNAREs in Neurodegenerative Diseases." Cells 10, no. 5 (2021): 991. http://dx.doi.org/10.3390/cells10050991.

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Neurodegenerative diseases are pathologies of the central and peripheral nervous systems characterized by loss of brain functions and problems in movement which occur due to the slow and progressive degeneration of cellular elements. Several neurodegenerative diseases are known such as Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis and many studies on the molecular mechanisms underlying these pathologies have been conducted. Altered functions of some key proteins and the presence of intraneuronal aggregates have been identified as responsible for the development of

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19

Джапаралиева, Н. Т., У. А. Нурбекова, Ж. М. Каримов та А. Ш. Шарабидинова. "Амиотрофиялык каптал склероздун үй-бүлөлүк формасынын генетикалык жана клиникалык аспектилери". Scientific and practical journal Healthcare of Kyrgyzstan, № 2 (10 травня 2024): 108–14. http://dx.doi.org/10.51350/zdravkg2024.2.6.15.108.114.

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РЕЗЮМЕ. Введение. Боковой амиатрофический склероз (БАС) – тяжелое редкое нейродегенеративное заболевание, которое отличается клинической и патогенетической гетерогенностью. Клиническая гетерогенность заключается в первоначальной локализации патологического процесса, вариабельности симптомов сочетанного поражения как верхнего, так и нижнего мотонейронов, возрасте дебюта, темпе прогрессирования, наследственной предрасположенности и наличии немоторной симптоматики. Ключевым методом постановки верного диагноза является также определение генов, ассоциированных с семейными формами болезни. Цель иссл

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20

Stoka, Veronika, Olga Vasiljeva, Hiroshi Nakanishi, and Vito Turk. "The Role of Cysteine Protease Cathepsins B, H, C, and X/Z in Neurodegenerative Diseases and Cancer." International Journal of Molecular Sciences 24, no. 21 (2023): 15613. http://dx.doi.org/10.3390/ijms242115613.

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Papain-like cysteine proteases are composed of 11 human cysteine cathepsins, originally located in the lysosomes. They exhibit broad specificity and act as endopeptidases and/or exopeptidases. Among them, only cathepsins B, H, C, and X/Z exhibit exopeptidase activity. Recently, cysteine cathepsins have been found to be present outside the lysosomes and often participate in various pathological processes. Hence, they have been considered key signalling molecules. Their potentially hazardous proteolytic activities are tightly regulated. This review aims to discuss recent advances in understandin

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21

Sheikh, Saba, Safia, Ejazul Haque, and Snober S. Mir. "Neurodegenerative Diseases: Multifactorial Conformational Diseases and Their Therapeutic Interventions." Journal of Neurodegenerative Diseases 2013 (December 30, 2013): 1–8. http://dx.doi.org/10.1155/2013/563481.

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Neurodegenerative diseases are multifactorial debilitating disorders of the nervous system that affect approximately 30 millionindividuals worldwide. Neurodegenerative diseases such as Alzheimer’s, Parkinson’s, Huntington’s, and amyotrophic lateral sclerosis diseases are the consequence of misfolding and dysfunctional trafficking of proteins. Beside that, mitochondrial dysfunction, oxidative stress, and/or environmental factors strongly associated with age have also been implicated in causing neurodegeneration. After years of intensive research, considerable evidence has accumulated that demon

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22

Kim, Haneul, Gon Sup Kim, Sang-Hwan Hyun, and Eunhye Kim. "Advancements in 2D and 3D In Vitro Models for Studying Neuromuscular Diseases." International Journal of Molecular Sciences 24, no. 23 (2023): 17006. http://dx.doi.org/10.3390/ijms242317006.

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Neuromuscular diseases (NMDs) are a genetically or clinically heterogeneous group of diseases that involve injury or dysfunction of neuromuscular tissue components, including peripheral motor neurons, skeletal muscles, and neuromuscular junctions. To study NMDs and develop potential therapies, remarkable progress has been made in generating in vitro neuromuscular models using engineering approaches to recapitulate the complex physical and biochemical microenvironments of 3D human neuromuscular tissues. In this review, we discuss recent studies focusing on the development of in vitro co-culture

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23

Winter, Aimee N., and Paula C. Bickford. "Anthocyanins and Their Metabolites as Therapeutic Agents for Neurodegenerative Disease." Antioxidants 8, no. 9 (2019): 333. http://dx.doi.org/10.3390/antiox8090333.

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Neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (ALS), are characterized by the death of neurons within specific regions of the brain or spinal cord. While the etiology of many neurodegenerative diseases remains elusive, several factors are thought to contribute to the neurodegenerative process, such as oxidative and nitrosative stress, excitotoxicity, endoplasmic reticulum stress, protein aggregation, and neuroinflammation. These processes culminate in the death of vulnerable neuronal populations, which manifests symptomaticall

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24

Huang, Jie, Mitchell Ringuet, Andrew E. Whitten, et al. "Structural basis of the zinc-induced cytoplasmic aggregation of the RNA-binding protein SFPQ." Nucleic Acids Research 48, no. 6 (2020): 3356–65. http://dx.doi.org/10.1093/nar/gkaa076.

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Abstract SFPQ is a ubiquitous nuclear RNA-binding protein implicated in many aspects of RNA biogenesis. Importantly, nuclear depletion and cytoplasmic accumulation of SFPQ has been linked to neuropathological conditions such as Alzheimer's disease (AD) and amyotrophic lateral sclerosis (ALS). Here, we describe a molecular mechanism by which SFPQ is mislocalized to the cytoplasm. We report an unexpected discovery of the infinite polymerization of SFPQ that is induced by zinc binding to the protein. The crystal structure of human SFPQ in complex with zinc at 1.94 Å resolution reveals intermolecu

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25

Gentile, Francesco, Pietro Emiliano Doneddu, Nilo Riva, Eduardo Nobile-Orazio, and Angelo Quattrini. "Diet, Microbiota and Brain Health: Unraveling the Network Intersecting Metabolism and Neurodegeneration." International Journal of Molecular Sciences 21, no. 20 (2020): 7471. http://dx.doi.org/10.3390/ijms21207471.

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Increasing evidence gives support for the idea that extra-neuronal factors may affect brain physiology and its predisposition to neurodegenerative diseases. Epidemiological and experimental studies show that nutrition and metabolic disorders such as obesity and type 2 diabetes increase the risk of Alzheimer’s and Parkinson’s diseases after midlife, while the relationship with amyotrophic lateral sclerosis is uncertain, but suggests a protective effect of features of metabolic syndrome. The microbiota has recently emerged as a novel factor engaging strong interactions with neurons and glia, dee

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Mathew, Angela Maria, Saatheeyavaane Bhuvanendran, Rajesh Sreedharan Nair, and Ammu K Radhakrishnan. "Exploring the anti-inflammatory activities, mechanism of action and prospective drug delivery systems of tocotrienol to target neurodegenerative diseases." F1000Research 12 (March 27, 2023): 338. http://dx.doi.org/10.12688/f1000research.131863.1.

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A major cause of death in the elderly worldwide is attributed to neurodegenerative diseases, such as AD (Alzheimer’s disease), PD (Parkinson’s disease), ALS (Amyotrophic lateral sclerosis), FRDA (Friedreich’s ataxia), VaD (Vascular dementia) etc. These can be caused due to multiple factors such as genetic, physiological problems like stroke or tumor, or even external causes like viruses, toxins, or chemicals. T3s (tocotrienols) exhibit various bioactive properties where it acts as an antioxidant, anti-inflammatory, anti-tumorigenic, and cholesterol lowering agent. Since T3 interferes with and

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27

Luís, João P., Carlos J. V. Simões, and Rui M. M. Brito. "The Therapeutic Prospects of Targeting IL-1R1 for the Modulation of Neuroinflammation in Central Nervous System Disorders." International Journal of Molecular Sciences 23, no. 3 (2022): 1731. http://dx.doi.org/10.3390/ijms23031731.

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The interleukin-1 receptor type 1 (IL-1R1) holds pivotal roles in the immune system, as it is positioned at the “epicenter” of the inflammatory signaling networks. Increased levels of the cytokine IL-1 are a recognized feature of the immune response in the central nervous system (CNS) during injury and disease, i.e., neuroinflammation. Despite IL-1/IL-1R1 signaling within the CNS having been the subject of several studies, the roles of IL-1R1 in the CNS cellular milieu still cause controversy. Without much doubt, however, the persistent activation of the IL-1/IL-1R1 signaling pathway is intima

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Cheng, Hei W. A., Timothy B. Callis, Andrew P. Montgomery, et al. "Understanding In Vitro Pathways to Drug Discovery for TDP-43 Proteinopathies." International Journal of Molecular Sciences 23, no. 23 (2022): 14769. http://dx.doi.org/10.3390/ijms232314769.

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The use of cellular models is a common means to investigate the potency of therapeutics in pre-clinical drug discovery. However, there is currently no consensus on which model most accurately replicates key aspects of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) pathology, such as accumulation of insoluble, cytoplasmic transactive response DNA-binding protein (TDP-43) and the formation of insoluble stress granules. Given this, we characterised two TDP-43 proteinopathy cellular models that were based on different aetiologies of disease. The first was a sodium arsenite-i

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29

Colantoni, Alessio, Davide Capauto, Vincenzo Alfano, et al. "FUS Alters circRNA Metabolism in Human Motor Neurons Carrying the ALS-Linked P525L Mutation." International Journal of Molecular Sciences 24, no. 4 (2023): 3181. http://dx.doi.org/10.3390/ijms24043181.

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Deregulation of RNA metabolism has emerged as one of the key events leading to the degeneration of motor neurons (MNs) in Amyotrophic Lateral Sclerosis (ALS) disease. Indeed, mutations on RNA-binding proteins (RBPs) or on proteins involved in aspects of RNA metabolism account for the majority of familiar forms of ALS. In particular, the impact of the ALS-linked mutations of the RBP FUS on many aspects of RNA-related processes has been vastly investigated. FUS plays a pivotal role in splicing regulation and its mutations severely alter the exon composition of transcripts coding for proteins inv

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Hsu, Sheng-Kai, Chien-Chih Chiu, Hans-Uwe Dahms, et al. "Unfolded Protein Response (UPR) in Survival, Dormancy, Immunosuppression, Metastasis, and Treatments of Cancer Cells." International Journal of Molecular Sciences 20, no. 10 (2019): 2518. http://dx.doi.org/10.3390/ijms20102518.

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The endoplasmic reticulum (ER) has diverse functions, and especially misfolded protein modification is in the focus of this review paper. With a highly regulatory mechanism, called unfolded protein response (UPR), it protects cells from the accumulation of misfolded proteins. Nevertheless, not only does UPR modify improper proteins, but it also degrades proteins that are unable to recover. Three pathways of UPR, namely PERK, IRE-1, and ATF6, have a significant role in regulating stress-induced physiological responses in cells. The dysregulated UPR may be involved in diseases, such as atheroscl

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31

Sévigny, Myriam, Isabelle Bourdeau Julien, Janani Priya Venkatasubramani, Jeremy B. Hui, Paul A. Dutchak, and Chantelle F. Sephton. "FUS contributes to mTOR-dependent inhibition of translation." Journal of Biological Chemistry 295, no. 52 (2020): 18459–73. http://dx.doi.org/10.1074/jbc.ra120.013801.

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The amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)–linked RNA-binding protein called FUS (fused in sarcoma) has been implicated in several aspects of RNA regulation, including mRNA translation. The mechanism by which FUS affects the translation of polyribosomes has not been established. Here we show that FUS can associate with stalled polyribosomes and that this association is sensitive to mTOR (mammalian target of rapamycin) kinase activity. Specifically, we show that FUS association with polyribosomes is increased by Torin1 treatment or when cells are cultured in nutri

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32

Damiano, Simona, Concetta Sozio, Giuliana La Rosa, et al. "Metabolism Regulation and Redox State: Insight into the Role of Superoxide Dismutase 1." International Journal of Molecular Sciences 21, no. 18 (2020): 6606. http://dx.doi.org/10.3390/ijms21186606.

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Energy metabolism and redox state are strictly linked; energy metabolism is a source of reactive oxygen species (ROS) that, in turn, regulate the flux of metabolic pathways. Moreover, to assure redox homeostasis, metabolic pathways and antioxidant systems are often coordinately regulated. Several findings show that superoxide dismutase 1 (SOD1) enzyme has effects that go beyond its superoxide dismutase activity and that its functions are not limited to the intracellular compartment. Indeed, SOD1 is secreted through unconventional secretory pathways, carries out paracrine functions and circulat

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33

Novellino, Fabiana, Valeria Saccà, Annalidia Donato, et al. "Innate Immunity: A Common Denominator between Neurodegenerative and Neuropsychiatric Diseases." International Journal of Molecular Sciences 21, no. 3 (2020): 1115. http://dx.doi.org/10.3390/ijms21031115.

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The intricate relationships between innate immunity and brain diseases raise increased interest across the wide spectrum of neurodegenerative and neuropsychiatric disorders. Barriers, such as the blood–brain barrier, and innate immunity cells such as microglia, astrocytes, macrophages, and mast cells are involved in triggering disease events in these groups, through the action of many different cytokines. Chronic inflammation can lead to dysfunctions in large-scale brain networks. Neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, amyotrophic la

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34

Zhang, Yiti, Baitao Zeng, Ao Gu, et al. "Damaged DNA Is an Early Event of Neurodegeneration in Induced Pluripotent Stem Cell-Derived Motoneurons with UBQLN2P497H Mutation." International Journal of Molecular Sciences 23, no. 19 (2022): 11333. http://dx.doi.org/10.3390/ijms231911333.

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Ubiquilin-2 (UBQLN2) mutations lead to familial amyotrophic lateral sclerosis (FALS)/and frontotemporal dementia (FTLD) through unknown mechanisms. The combination of iPSC technology and CRISPR-mediated genome editing technology can generate an iPSC-derived motor neuron (iPSC-MN) model with disease-relevant mutations, which results in increased opportunities for disease mechanism research and drug screening. In this study, we introduced a UBQLN2-P497H mutation into a healthy control iPSC line using CRISPR/Cas9, and differentiated into MNs to study the pathology of UBQLN2-related ALS. Our in vi

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Rahic, Zerina, Emanuele Buratti, and Sara Cappelli. "Reviewing the Potential Links between Viral Infections and TDP-43 Proteinopathies." International Journal of Molecular Sciences 24, no. 2 (2023): 1581. http://dx.doi.org/10.3390/ijms24021581.

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Transactive response DNA binding protein 43 kDa (TDP-43) was discovered in 2001 as a cellular factor capable to inhibit HIV-1 gene expression. Successively, it was brought to new life as the most prevalent RNA-binding protein involved in several neurological disorders, such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Despite the fact that these two research areas could be considered very distant from each other, in recent years an increasing number of publications pointed out the existence of a potentially important connection. Indeed, the ability of TD

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Srinivasan, Venkataramanan, Victoria Homer, Darren Barton, et al. "A low molecular weight dextran sulphate, ILB®, for the treatment of amyotrophic lateral sclerosis (ALS): An open-label, single-arm, single-centre, phase II trial." PLOS ONE 19, no. 7 (2024): e0291285. http://dx.doi.org/10.1371/journal.pone.0291285.

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Background Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig´s disease, is a rare neurological condition and is the most common motor neurone disease. It is a fatal disease with specific loss of motor neurons in the spinal cord, brain stem, and motor cortex leading to progressive paralysis and usually death within five years of diagnosis. There remains no cure for ALS, and management is focused on a combination of neuroprotective medication, respiratory support, and management by multidisciplinary clinics. Patients and methods This prospective, single-arm, open-label phase II clini

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Bampton, Alexander, Ariana Gatt, Jack Humphrey, et al. "HnRNP K mislocalisation is a novel protein pathology of frontotemporal lobar degeneration and ageing and leads to cryptic splicing." Acta Neuropathologica 142, no. 4 (2021): 609–27. http://dx.doi.org/10.1007/s00401-021-02340-0.

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AbstractHeterogeneous nuclear ribonucleoproteins (HnRNPs) are a group of ubiquitously expressed RNA-binding proteins implicated in the regulation of all aspects of nucleic acid metabolism. HnRNP K is a member of this highly versatile hnRNP family. Pathological redistribution of hnRNP K to the cytoplasm has been linked to the pathogenesis of several malignancies but, until now, has been underexplored in the context of neurodegenerative disease. Here we show hnRNP K mislocalisation in pyramidal neurons of the frontal cortex to be a novel neuropathological feature that is associated with both fro

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Bursch, Franziska, Norman Kalmbach, Maximilian Naujock, et al. "Altered calcium dynamics and glutamate receptor properties in iPSC-derived motor neurons from ALS patients with C9orf72, FUS, SOD1 or TDP43 mutations." Human Molecular Genetics 28, no. 17 (2019): 2835–50. http://dx.doi.org/10.1093/hmg/ddz107.

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Abstract The fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS) is characterized by a profound loss of motor neurons (MNs). Until now only riluzole minimally extends life expectancy in ALS, presumably by inhibiting glutamatergic neurotransmission and calcium overload of MNs. Therefore, the aim of this study was to investigate the glutamate receptor properties and key aspects of intracellular calcium dynamics in induced pluripotent stem cell (iPSC)-derived MNs from ALS patients with C9orf72 (n = 4 cell lines), fused in sarcoma (FUS) (n = 9), superoxide dismutase 1 (SOD1) (n = 3

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Yang, Chunxing, Tao Qiao, Jia Yu, et al. "Low-level overexpression of wild type TDP-43 causes late-onset, progressive neurodegeneration and paralysis in mice." PLOS ONE 17, no. 2 (2022): e0255710. http://dx.doi.org/10.1371/journal.pone.0255710.

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Modestly increased expression of transactive response DNA binding protein (TDP-43) gene have been reported in amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and other neuromuscular diseases. However, whether this modest elevation triggers neurodegeneration is not known. Although high levels of TDP-43 overexpression have been modeled in mice and shown to cause early death, models with low-level overexpression that mimic the human condition have not been established. In this study, transgenic mice overexpressing wild type TDP-43 at less than 60% above the endogenous CNS leve

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Rodrigues, Maria Carolina O., Júlio C. Voltarelli, Paul R. Sanberg, Cesario V. Borlongan, and Svitlana Garbuzova-Davis. "Immunological Aspects in Amyotrophic Lateral Sclerosis." Translational Stroke Research 3, no. 3 (2012): 331–40. http://dx.doi.org/10.1007/s12975-012-0177-6.

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Gulino, Rosario, Nunzio Vicario, Maria Giunta, et al. "Neuromuscular Plasticity in a Mouse Neurotoxic Model of Spinal Motoneuronal Loss." International Journal of Molecular Sciences 20, no. 6 (2019): 1500. http://dx.doi.org/10.3390/ijms20061500.

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Despite the relevant research efforts, the causes of amyotrophic lateral sclerosis (ALS) are still unknown and no effective cure is available. Many authors suggest that ALS is a multi-system disease caused by a network failure instead of a cell-autonomous pathology restricted to motoneurons. Although motoneuronal loss is the critical hallmark of ALS given their specific vulnerability, other cell populations, including muscle and glial cells, are involved in disease onset and progression, but unraveling their specific role and crosstalk requires further investigation. In particular, little is k

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Voitenkov, Vladislav B., and E. V. Ekusheva. "Pain in amyotrophic lateral sclerosis." Journal of Clinical Practice 10, no. 2 (2019): 66–73. http://dx.doi.org/10.17816/clinpract10266-73.

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In this review, we discuss different aspects of pain syndrome in patients with amyotrophic lateral sclerosis: etiology, incidence, pathophysiology and main clinical features. Also we review the modern approaches to the treatment of pain in amyotrophic lateral sclerosis. Pain is actually not rare in this condition: it appears in 80% of patients, affecting their quality of life and functional activity, leading to the development of depressive and anxiety disorders. Pain in amyotrophic lateral sclerosis is often overlooked by clinicians, since their attention may focus on the motor symptoms of th

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Belleroche, J. De, R. W. Orrell, and L. Virgo. "Amyotrophic Lateral Sclerosis." Journal of Neuropathology and Experimental Neurology 55, no. 7 (1996): 747–57. http://dx.doi.org/10.1097/00005072-199607000-00001.

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OBERLEY, LARRY W. "Amyotrophic Lateral Sclerosis." Journal of Neuropathology and Experimental Neurology 56, no. 4 (1997): 455. http://dx.doi.org/10.1097/00005072-199704000-00015.

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Alekseeva, T. M., V. S. Demeshonok, and S. N. Zhulev. "Deontological aspects of the amyotrophic lateral sclerosis." Neuromuscular Diseases 7, no. 4 (2017): 56–60. http://dx.doi.org/10.17650/2222-8721-2017-7-4-56-60.

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Krivickas, Lisa S., and Gregory T. Carter. "Amyotrophic Lateral Sclerosis: Practical Aspects Of Care." Journal of Spinal Cord Medicine 25, no. 4 (2002): 274–76. http://dx.doi.org/10.1080/10790268.2002.11753627.

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Norris, Lorenzo, Guinevere Que, and Elham Bayat. "Psychiatric Aspects of Amyotrophic Lateral Sclerosis (ALS)." Current Psychiatry Reports 12, no. 3 (2010): 239–45. http://dx.doi.org/10.1007/s11920-010-0118-6.

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Hu, Yushu, Wenzhi Chen, Caihui Wei, et al. "Pathological mechanisms of amyotrophic lateral sclerosis." Neural Regeneration Research 19, no. 5 (2023): 1036–44. http://dx.doi.org/10.4103/1673-5374.382985.

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Abstract Amyotrophic lateral sclerosis refers to a neurodegenerative disease involving the motor system, the cause of which remains unexplained despite several years of research. Thus, the journey to understanding or treating amyotrophic lateral sclerosis is still a long one. According to current research, amyotrophic lateral sclerosis is likely not due to a single factor but rather to a combination of mechanisms mediated by complex interactions between molecular and genetic pathways. The progression of the disease involves multiple cellular processes and the interaction between different comp

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Hand, Collette K., and Guy A. Rouleau. "Familial amyotrophic lateral sclerosis." Muscle & Nerve 25, no. 2 (2002): 135–59. http://dx.doi.org/10.1002/mus.10001.

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Souza, Paulo Victor Sgobbi de, Paulo de Lima Serrano, Igor Braga Farias, et al. "Clinical and Genetic Aspects of Juvenile Amyotrophic Lateral Sclerosis: A Promising Era Emerges." Genes 15, no. 3 (2024): 311. http://dx.doi.org/10.3390/genes15030311.

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Juvenile Amyotrophic Lateral Sclerosis is a genetically heterogeneous neurodegenerative disorder, which is frequently misdiagnosed due to low clinical suspicion and little knowledge about disease characteristics. More than 20 different genetic loci have been associated with both sporadic and familial juvenile Amyotrophic Lateral Sclerosis. Currently, almost 40% of cases have an identifiable monogenic basis; type 6, associated with FUS gene variants, is the most prevalent globally. Despite several upper motor neuron-dominant forms being generally associated with long-standing motor symptoms and

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