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Amyotrophic Lateral Sclerosis

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Carbonic anhydrase IX as a novel candidate in liquid biopsy.

Ozen Ozensoy Guler, Claudiu T Supuran, Clemente Capasso,

Among the diagnostic techniques for the identification of tumour biomarkers, the liquid biopsy is considered one that offers future research on precision diagnosis and treatment of tumours in a non-invasive manner. The approach consists of isolating tumor-derived components, such as circulating tumour cells (CTC), tumour cell-free DNA (ctDNA), and extracellular ... Read more >>

J Enzyme Inhib Med Chem (Journal of enzyme inhibition and medicinal chemistry)
[2020, 35(1):255-260]

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Benzothiazole derivatives as anticancer agents.

Ali Irfan, Fozia Batool, Syeda Andleeb Zahra Naqvi, Amjad Islam, Sameh M Osman, Alessio Nocentini, Siham A Alissa, Claudiu T Supuran,

Benzothiazole (BTA) belongs to the heterocyclic class of bicyclic compounds. BTA derivatives possesses broad spectrum biological activities such as anticancer, antioxidant, anti-inflammatory, anti-tumour, antiviral, antibacterial, anti-proliferative, anti-diabetic, anti-convulsant, analgesic, anti-tubercular, antimalarial, anti-leishmanial, anti-histaminic and anti-fungal among others. The BTA scaffolds showed a crucial role in the inhibition of the metalloenzyme ... Read more >>

J Enzyme Inhib Med Chem (Journal of enzyme inhibition and medicinal chemistry)
[2020, 35(1):265-279]

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New insights into Wnt signaling alterations in amyotrophic lateral sclerosis: a potential therapeutic target?

Carlos Gonzalez-Fernandez, Pau González, Francisco Javier Rodríguez,

Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder characterized by upper and lower motor neuron degeneration, which leads to progressive paralysis of skeletal muscles and, ultimately, respiratory failure between 2-5 years after symptom onset. Unfortunately, currently accepted treatments for amyotrophic lateral sclerosis are extremely scarce and only provide modest benefit. ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(9):1580-1589]

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Understanding metabolic flexibility: a potential key to unlocking metabolic therapies in amyotrophic lateral sclerosis?

Scott P Allen,

Neural Regen Res (Neural regeneration research)
[2020, 15(9):1654-1655]

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The roles of atypical protein kinase Cs (aPKCs) in the nervous system: targets for neuroregeneration?

Dennis Dik-Long Chau, Kwok-Fai Lau,

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1475-1476]

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Exercise promotes recovery after motoneuron injury via hormonal mechanisms.

Cory Chew, Dale R Sengelaub,

Injuries to spinal motoneurons manifest in a variety of forms, including damage to peripheral axons, neurodegenerative disease, or direct insult centrally. Such injuries produce a variety of negative structural and functional changes in both the directly affected and neighboring motoneurons. Exercise is a relatively simple behavioral intervention that has been ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1373-1376]

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Type XIX collagen: a promising biomarker from the basement membranes.

Ana C Calvo, Laura Moreno, Leticia Moreno, Janne M Toivonen, Raquel Manzano, Nora Molina, Miriam de la Torre, Tresa López, Francisco J Miana-Mena, María J Muñoz, Pilar Zaragoza, Pilar Larrodé, Alberto García-Redondo, Rosario Osta,

Among collagen members in the collagen superfamily, type XIX collagen has raised increasing interest in relation to its structural and biological roles. Type XIX collagen is a Fibril-Associated Collagen with Interrupted Triple helices member, one main subclass of collagens in this superfamily. This collagen contains a triple helix composed of ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(6):988-995]

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Novel small molecule TRVA242 targets neuromuscular junction in amyotrophic lateral sclerosis.

Poulomee Bose,

Neural Regen Res (Neural regeneration research)
[2020, 15(6):1041-1042]

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Biological characteristics of dynamic expression of nerve regeneration related growth factors in dorsal root ganglia after peripheral nerve injury.

Yin-Ying Shen, Xiao-Kun Gu, Rui-Rui Zhang, Tian-Mei Qian, Shi-Ying Li, Sheng Yi,

The regenerative capacity of peripheral nerves is limited after nerve injury. A number of growth factors modulate many cellular behaviors, such as proliferation and migration, and may contribute to nerve repair and regeneration. Our previous study observed the dynamic changes of genes in L4-6 dorsal root ganglion after rat sciatic ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1502-1509]

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The N-formyl peptide receptors: contemporary roles in neuronal function and dysfunction.

Peter J G Cussell, Margarita Gomez Escalada, Nathaniel G N Milton, Andrew W J Paterson,

N-formyl peptide receptors (FPRs) were first identified upon phagocytic leukocytes, but more than four decades of research has unearthed a plethora of non-myeloid roles for this receptor family. FPRs are expressed within neuronal tissues and markedly in the central nervous system, where FPR interactions with endogenous ligands have been implicated ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(7):1191-1198]

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Aggressive FUS-Mutant Motor Neuron Disease Without Profound Spinal Cord Pathology.

Yan Chen Wongworawat, Yin Allison Liu, Ravi Raghavan, Charles L White, Robin Dietz, Craig Zuppan, Jeffrey Rosenfeld,

A 29-year-old man presented with rapidly progressive severe neck weakness, asymmetrical bilateral upper extremity weakness, bulbar dysfunction, profound muscle wasting, and weight loss. Within 1 year, his speech became unintelligible, he became gastrostomy- and tracheostomy/ventilator-dependent, and wheelchair bound. Electrophysiology suggested motor neuron disease. Whole exome sequencing revealed a heterozygous pathogenic ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):365-369]

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Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis.

Kensuke Ikenaka, Shinsuke Ishigaki, Yohei Iguchi, Kaori Kawai, Yusuke Fujioka, Satoshi Yokoi, Rehab F Abdelhamid, Seiichi Nagano, Hideki Mochizuki, Masahisa Katsuno, Gen Sobue,

Alterations of RNA metabolism caused by mutations in RNA-binding protein genes, such as transactivating DNA-binding protein-43 (TDP-43) and fused in sarcoma (FUS), have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). Unlike the accumulation of TDP43, which is accepted as a pathological hall mark of sporadic ALS (sALS), ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):370-377]

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The Role of Sex and Sex Hormones in Neurodegenerative Diseases.

Elisabetta Vegeto, Alessandro Villa, Sara Della Torre, Valeria Crippa, Paola Rusmini, Riccardo Cristofani, Mariarita Galbiati, Adriana Maggi, Angelo Poletti,

Neurodegenerative diseases (NDs) are a wide class of disorders of the central nervous system (CNS) with unknown etiology. Several factors were hypothesized to be involved in the pathogenesis of these diseases, including genetic and environmental factors. Many of these diseases show a sex prevalence and sex steroids were shown to ... Read more >>

Endocr. Rev. (Endocrine reviews)
[2020, 41(2):]

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Lipids, apolipoproteins, and prognosis of amyotrophic lateral sclerosis.

Caroline Ingre, Lin Chen, Yiqiang Zhan, Jet Termorshuizen, Li Yin, Fang Fang,

OBJECTIVE:To determine whether lipids and apolipoproteins predict prognosis of patients with amyotrophic lateral sclerosis in a cohort study of 99 patients with amyotrophic lateral sclerosis who were diagnosed during 2015 to 2018 and followed up until October 31, 2018, at the Neurology Clinic in Karolinska University Hospital in Stockholm, Sweden. ... Read more >>

Neurology (Neurology)
[2020, :]

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The multifaceted role of kinases in amyotrophic lateral sclerosis: genetic, pathological and therapeutic implications.

Wenting Guo, Tijs Vandoorne, Jolien Steyaert, Kim A Staats, Ludo Van Den Bosch,

Amyotrophic lateral sclerosis is the most common degenerative disorder of motor neurons in adults. As there is no cure, thousands of individuals who are alive at present will succumb to the disease. In recent years, numerous causative genes and risk factors for amyotrophic lateral sclerosis have been identified. Several of ... Read more >>

Brain (Brain : a journal of neurology)
[2020, :]

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Amyotrophic lateral sclerosis with coexisting cancer: a single-center study.

Onur Akan, Leyla Baysal-Kirac,

This study investigated the occurrence of malignancy in a cohort of patients with amyotrophic lateral sclerosis (ALS). Forty-three consecutive ALS patients (mean age 61.3 years, 31 men/12 women) admitted between 2015 and 2019 were enrolled. Clinical, electrophysiologic and outcome features that differentiate cancer-associated ALS from classical ALS were investigated. ALS was ... Read more >>

Acta Neurol Belg (Acta neurologica Belgica)
[2020, :]

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The joint lasso: high-dimensional regression for group structured data.

Frank Dondelinger, Sach Mukherjee, ,

We consider high-dimensional regression over subgroups of observations. Our work is motivated by biomedical problems, where subsets of samples, representing for example disease subtypes, may differ with respect to underlying regression models. In the high-dimensional setting, estimating a different model for each subgroup is challenging due to limited sample sizes. ... Read more >>

Biostatistics (Biostatistics (Oxford, England))
[2020, 21(2):219-235]

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Regional brain susceptibility to neurodegeneration: what is the role of glial cells?

Andrea Beatriz Cragnolini, Giorgia Lampitella, Assunta Virtuoso, Immacolata Viscovo, Fivos Panetsos, Michele Papa, Giovanni Cirillo,

The main pathological feature of the neurodegenerative diseases is represented by neuronal death that represents the final step of a cascade of adverse/hostile events. Early in the neurodegenerative process, glial cells (including astrocytes, microglial cells, and oligodendrocytes) activate and trigger an insidious neuroinflammatory reaction, metabolic decay, blood brain barrier dysfunction ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):838-842]

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Primary lateral sclerosis: diagnosis and management.

Martin R Turner, Kevin Talbot,

Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder at the upper motor neurone extreme of the spectrum of motor neurone disease. The diagnosis is clinical and based on the characteristic features of slowly progressive spasticity beginning in the lower limbs, or more rarely with spastic dysarthria, typically presenting around ... Read more >>

Pract Neurol (Practical neurology)
[2020, :]

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Improving Access to Clinical Trials for Amyotrophic Lateral Sclerosis Treatment.

Maya Vijayaraghavan,

JAMA Neurol (JAMA neurology)
[2020, :]

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Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis.

J Bashford, U Masood, A Wickham, R Iniesta, E Drakakis, M Boutelle, K Mills, C Shaw,

BACKGROUND:Fasciculations represent early neuronal hyperexcitability in amyotrophic lateral sclerosis (ALS). To aid calibration as a disease biomarker, we set out to characterise the daytime variability of fasciculation firing. METHODS:Fasciculation awareness scores were compiled from 19 ALS patients. Additionally, ten ALS patients prospectively underwent high-density surface EMG (HDSEMG) recordings from biceps ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, :]

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Structural basis of the zinc-induced cytoplasmic aggregation of the RNA-binding protein SFPQ.

Jie Huang, Mitchell Ringuet, Andrew E Whitten, Sofia Caria, Yee Wa Lim, Rahul Badhan, Victor Anggono, Mihwa Lee,

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 ... Read more >>

Nucleic Acids Res. (Nucleic acids research)
[2020, 48(6):3356-3365]

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A mixtures approach to solvent exposures and amyotrophic lateral sclerosis: a population-based study in Denmark.

Aisha S Dickerson, Johnni Hansen, Shiraya Thompson, Ole Gredal, Marc G Weisskopf,

Studies of occupational solvent exposures and amyotrophic lateral sclerosis (ALS) have been conflicting. We conducted a population-based case-control study of mixed occupational solvent exposures and ALS. Using the Danish National Patient Registry, we identified ALS cases in Denmark from 1982 to 2013, and matched them to 100 controls based on ... Read more >>

Eur. J. Epidemiol. (European journal of epidemiology)
[2020, :]

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A mimetic peptide of α2,6-sialyllactose promotes neuritogenesis.

Shuang-Xi Chen, Jia-Hui He, Yong-Jian Mi, Hui-Fan Shen, Melitta Schachner, Wei-Jiang Zhao,

Oxidative stress contributes to the pathogenesis of neurodegenerative diseases. With the aim to find reagents that reduce oxidative stress, a phage display library was screened for peptides mimicking α2,6-sialyllactose (6'-SL), which is known to beneficially influence neural functions. Using Sambucus nigra lectin, which specifically binds to 6'-SL, we screened a ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(6):1058-1065]

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The transcription factor Nurr1 is up-regulated in amyotrophic lateral sclerosis patients and SOD1-G93A mice.

Valeria Valsecchi, Marina Boido, Francesca Montarolo, Michela Guglielmotto, Simona Perga, Serena Martire, Santina Cutrupi, Andrea Iannello, Nadia Gionchiglia, Elena Signorino, Andrea Calvo, Giuseppe Fuda, Adriano Chiò, Antonio Bertolotto, Alessandro Vercelli,

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects both lower and upper motor neurons (MNs) in the central nervous system (CNS). ALS etiology is highly multifactorial and multifarious, and an effective treatment is still lacking. Neuroinflammation is a hallmark of ALS and could be targeted to develop new ... Read more >>

Dis Model Mech (Disease Models & Mechanisms)
[2020, :]

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