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Primary 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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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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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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VRK1 variants in two Portuguese unrelated patients with childhood-onset motor neuron disease.

Daniela Pimenta Silva, Mariana Soeiro E Sá, Fernando Silveira, Susana Pinto, Marta Gromicho, Ana Berta Sousa, Miguel Leão, Mamede De Carvalho,

VRK1 encodes a serine/protein kinase possibly involved in pathways related to amyotrophic lateral sclerosis (ALS) pathogenesis. Pathogenic variants in VRK1 have been related to different phenotypes. We describe the clinical phenotype of two unrelated Portuguese patients with different VRK1 variants. Both patients presented a bilateral distal weakness in lower limbs ... Read more >>

Amyotroph Lateral Scler Frontotemporal Degener (Amyotrophic lateral sclerosis & frontotemporal degeneration)
[2020, :1-5]

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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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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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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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A robust and novel electrical impedance metric of pulmonary function in ALS patients.

Badria Munir, Ethan K Murphy, Akashleena Mallick, Hilda Gutierrez, Fu Zhang, Sarah Verga, Christy Smith, Sean D Levy, Courtney McIlduff, Sarbesh Pandeya, Ryan J Halter, Seward B Rutkove,

OBJECTIVE:Pulmonary function tests (PFTs) are important for assessing respiratory function in amyotrophic lateral sclerosis (ALS) patients. However, weakness of oral and glottal closure, due to concomitant bulbar dysfunction, may result in unreliable PFT values stemming from leakage of air around the breathing tube and through the glottis. In this study, ... Read more >>

Physiol Meas (Physiological measurement)
[2020, :]

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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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Expression of ALS-linked SOD1 mutation in motoneurons or myotubes induces differential effects on neuromuscular function in vitro.

Salim Benlefki, Ana Sanchez-Vicente, Vanessa Milla, Olivier Lucas, Claire Soulard, Richard Younes, Csilla Gergely, Mélissa Bowerman, Cédric Raoul, Frédérique Scamps, Cécile Hilaire,

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that selectively affects upper and lower motoneurons. Dismantlement of the neuromuscular junction (NMJ) is an early pathological hallmark of the disease whose cellular origin remains still debated. We developed an in vitro NMJ model to investigate the differential contribution of motoneurons ... Read more >>

Neuroscience (Neuroscience)
[2020, :]

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Osmotic pressure of serum and cerebrospinal fluid in patients with suspected neurological conditions.

Tetsuya Akaishi, Toshiyuki Takahashi, Ichiro Nakashima, Michiaki Abe, Masashi Aoki, Tadashi Ishii,

Interstitial fluid movement in the brain parenchyma has been suggested to contribute to sustaining the metabolism in brain parenchyma and maintaining the function of neurons and glial cells. The pulsatile hydrostatic pressure gradient may be one of the driving forces of this bulk flow. However, osmotic pressure-related factors have not ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):944-947]

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Differentiating Flail Limb Syndrome from Amyotrophic Lateral Sclerosis.

Jeffrey Kornitzer, Hael F Abdulrazeq, Mohammad Zaidi, John R Bach, Abdul Kazi, Erin Feinstein, Howard W Sander, Nizar Souayah,

OBJECTIVE:To investigate differences between flail limb syndrome (FLS) and amyotrophic lateral sclerosis (ALS). DESIGN:Retrospective chart review identified 16 cases of ALS and 16 of FLS. Revised ALS Functional Rating Scale (ALSFRS-R), compound muscle action potential (CMAP) amplitudes, and rate of loss of vital capacity (ΔVC) were compared. RESULTS:Comparing ALS and ... Read more >>

Am J Phys Med Rehabil (American journal of physical medicine & rehabilitation)
[2020, :]

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Edible seaweed-derived constituents: an undisclosed source of neuroprotective compounds.

Melissa Schepers, Nikita Martens, Assia Tiane, Kenneth Vanbrabant, Hong-Bing Liu, Dieter Lütjohann, Monique Mulder, Tim Vanmierlo,

Edible marine algae, or seaweeds, are a rich source of several bioactive compounds including phytosterols, carotenoids, and polysaccharides. Over the last decades, seaweed-derived constituents turned out to not only reside in the systemic circulation, but are able to cross the blood-brain barrier to exert neuro-active functions both in homeostatic and ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):790-795]

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Why do anti-inflammatory signals of bone marrow-derived stromal cells improve neurodegenerative conditions where anti-inflammatory drugs fail?

J P J M de Munter, J Mey, T Strekalova, B W Kramer, E Ch Wolters,

Neurodegenerative disorders share the final degenerative pathway, the inflammation-induced apoptosis and/or necrosis, irrespective of their etiology, be it of acute and chronic traumatic, vascular and idiopathic origin. Although disease-modifying strategies are an unmet need in these disorders, lately, (pre)clinical studies suggested favorable effects after an intervention with bone marrow-derived stromal ... Read more >>

J Neural Transm (Vienna) (Journal of neural transmission (Vienna, Austria : 1996))
[2020, :]

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The relationship between selected sociodemographic factors and speech organ dysfunction in sporadic ALS patients

Wioletta Pawlukowska, Bartłomiej Baumert, Monika Gołąb-Janowska, Agnieszka Meller, Karolina Machowska-Sempruch, Agnieszka Wełnicka, Edyta Paczkowska, Iwona Rotter, Bogusław Machaliński, Przemysław Nowacki,

<title>Abstract</title> <p> <bold>Background: </bold>Speech disorders are observed in 30% of newly diagnosed sporadic amyotrophic lateral sclerosis (ALS) patients. Characterized by a dynamic course, dysfunction of articulation has not so far been well understood. The aim of this study was to analyze the influence of sociodemographic factors (sex, age, ... Read more >>

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Omics Approach to Axonal Dysfunction of Motor Neurons in Amyotrophic Lateral Sclerosis (ALS).

Naoki Suzuki, Tetsuya Akiyama, Hitoshi Warita, Masashi Aoki,

Amyotrophic lateral sclerosis (ALS) is an intractable adult-onset neurodegenerative disease that leads to the loss of upper and lower motor neurons (MNs). The long axons of MNs become damaged during the early stages of ALS. Genetic and pathological analyses of ALS patients have revealed dysfunction in the MN axon homeostasis. ... Read more >>

Front Neurosci (Frontiers in Neuroscience)
[2020, 14:194]

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Protein transmission in neurodegenerative disease.

Chao Peng, John Q Trojanowski, Virginia M-Y Lee,

Most neurodegenerative diseases are characterized by the intracellular or extracellular aggregation of misfolded proteins such as amyloid-β and tau in Alzheimer disease, α-synuclein in Parkinson disease, and TAR DNA-binding protein 43 in amyotrophic lateral sclerosis. Accumulating evidence from both human studies and disease models indicates that intercellular transmission and the ... Read more >>

Nat Rev Neurol (Nature reviews. Neurology)
[2020, 16(4):199-212]

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Generation of twelve induced pluripotent stem cell lines from two healthy controls and two patients with sporadic amyotrophic lateral sclerosis.

Meimei Yang, Min Liu, Yicheng Ding, Alice Vajda, Jun Ma, Huixian Cui, Timothy O'Brien, David Henshall, Orla Hardiman, Sanbing Shen,

The majority of amyotrophic lateral sclerosis are sporadic (sALS) with no familial history or known genetic association, therefore a large cohort of disease models are required to identify common mechanisms or to test therapeutic interventions. Here we generated twelve induced pluripotent stem cell (iPSC) lines from human dermal fibroblasts of ... Read more >>

Stem Cell Res (Stem cell research)
[2020, 44:101752]

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Glucose, glycolysis, and neurodegenerative diseases.

Bor Luen Tang,

Prolonged survival of a typical postmitotic neuron hinges on a balance between multiple processes, among these are a sustenance of ATP production and protection against reactive oxygen species. In neuropathological conditions, mitochondrial defects often lead to both a drop in ATP levels, as well as increase reactive oxygen species production ... Read more >>

J. Cell. Physiol. (Journal of cellular physiology)
[2020, :]

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Exosomes as mediators of neuron-glia communication in neuroinflammation.

María Pascual, Francesc Ibáñez, Consuelo Guerri,

In recent years, a type of extracellular vesicles named exosomes has emerged that play an important role in intercellular communication under physiological and pathological conditions. These nanovesicles (30-150 nm) contain proteins, RNAs and lipids, and their internalization by bystander cells could alter their normal functions. This review focuses on recent ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):796-801]

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Klotho Pathways, Myelination Disorders, Neurodegenerative Diseases, and Epigenetic Drugs.

Walter H Moos, Douglas V Faller, Ioannis P Glavas, David N Harpp, Iphigenia Kanara, Anastasios N Mavrakis, Julie Pernokas, Mark Pernokas, Carl A Pinkert, Whitney R Powers, Konstantina Sampani, Kosta Steliou, Demetrios G Vavvas, Robert J Zamboni, Krishna Kodukula, Xiaohong Chen,

In this review we outline a rationale for identifying neuroprotectants aimed at inducing endogenous Klotho activity and expression, which is epigenetic action, by definition. Such an approach should promote remyelination and/or stimulate myelin repair by acting on mitochondrial function, thereby heralding a life-saving path forward for patients suffering from neuroinflammatory ... Read more >>

Biores Open Access (BioResearch open access)
[2020, 9(1):94-105]

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IN VITRO AND IN VIVO MODELS OF AMYOTROPHIC LATERAL SCLEROSIS: AN UPDATED OVERVIEW.

Auderlan M Gois, Deise M F Mendonça, Marco Aurelio M Freire, Jose R Santos,

Amyotrophic Lateral Sclerosis (ALS) is a progressive, neurodegenerative disease characterized by loss of upper motor neurons (UMN) and lower motor neurons (LMN). Disease affects people all over the world and is more prevalent in men. Patients with ALS develop extensive muscle wasting, paralysis and ultimately death, with a median survival ... Read more >>

Brain Res. Bull. (Brain research bulletin)
[2020, 159:32-43]

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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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