Neurofilament Subunit L Levels in the Cerebrospinal Fluid and Serum of Patients with Amyotrophic Lateral Sclerosis

Conclusions: NF-L levels increased in CSF and serum of patients with ALS. NF-L may thus be a neurodegenerative biomarker for predicting ALS severity and progression, and the survival of patients with this disease.Neurodegener Dis
Source: Neurodegenerative Diseases - Category: Neurology Source Type: research

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This article discusses the preclinical pharmacology, pharmacokinetics, safety profile, clinical studies and drug interactions of edaravone (Radicava) in ALS. PMID: 29998226 [PubMed - in process]
Source: Drugs of Today - Category: Drugs & Pharmacology Tags: Drugs Today (Barc) Source Type: research
There are several suspected infectious causes of amyotrophic lateral sclerosis (ALS) or motor neurone disease including HIV-1 and species of Brucella, Cyanobacteria and Schistosoma. The increased rates and clusters of ALS in amateur and professional outdoor sports players including rugby, football and soccer players suggest a microorganism present in the grass, dirt and dust they play on and in may be a causative factor. The probable zoonosis Mycobacterium avium subspecies paratuberculosis (MAP) is heavily excreted in an infected domestic ruminant ’s feces or manure and is extensively distributed throughout the soil ...
Source: Medical Hypotheses - Category: Biomedical Science Authors: Source Type: research
Publication date: Available online 19 June 2018Source: The Lancet NeurologyAuthor(s): Albert C Ludolph, Joachim Schuster, Johannes Dorst, Luc Dupuis, Jens Dreyhaupt, Jochen H Weishaupt, Jan Kassubek, Ulrike Weiland, Susanne Petri, Thomas Meyer, Julian Grosskreutz, Berthold Schrank, Matthias Boentert, Alexander Emmer, Andreas Hermann, Daniel Zeller, Johannes Prudlo, Andrea S Winkler, Torsten Grehl, Michael T HenekaSummaryBackgroundRasagiline, a monoamine oxidase B inhibitor with neuroprotective potential in Parkinson's disease, has shown a disease-modifying effect in the SOD1-Gly93Ala low-expressing mouse model of amyotroph...
Source: The Lancet Neurology - Category: Neurology Source Type: research
We report that treatment of hSOD1G93A mice with a selective KCa3.1 inhibitor, 1-[(2-chlorophenyl)diphenylmethyl]-1H-pyrazole (TRAM-34), attenuates the “pro-inflammatory” phenotype of microglia in the spinal cord, reduces motor neuron death, delays onset of muscle weakness, and increases survival. Specifically, inhibition of KCa3.1 channels slowed muscle denervation, decreased the expression of the fetal acetylcholine receptor γ subunit and reduced neuromuscular junction damage. Taken together, these results demonstrate a key role for KCa3.1 in driving a pro-inflammatory microglia phenotype in ALS.
Source: Brain, Behavior, and Immunity - Category: Neurology Source Type: research
Publication date: Available online 20 June 2018Source: Molecular ImmunologyAuthor(s): Anne-Lene Kjældgaard, Katrine Pilely, Karsten Skovgaard Olsen, Stephen Wørlich Pedersen, Anne Øberg Lauritsen, Kirsten Møller, Peter GarredAbstractAmyotrophic lateral sclerosis (ALS) is a devastating, neurodegenerative motor neuron disease. The aetiology of ALS remains an enigma which hinders the design of an effective treatment to prevent, postpone, or reverse the pathophysiological changes occurring during the aggressive progression of this disease.During the last decade, basic research within the innate immun...
Source: Molecular Immunology - Category: Allergy & Immunology Source Type: research
Publication date: 5 June 2018Source: Stem Cell Reports, Volume 10, Issue 6Author(s): Evangelos Kiskinis, Joel M. Kralj, Peng Zou, Eli N. Weinstein, Hongkang Zhang, Konstantinos Tsioras, Ole Wiskow, J. Alberto Ortega, Kevin Eggan, Adam E. CohenSummaryHuman induced pluripotent stem cell (iPSC)-derived neurons are an attractive substrate for modeling disease, yet the heterogeneity of these cultures presents a challenge for functional characterization by manual patch-clamp electrophysiology. Here, we describe an optimized all-optical electrophysiology, “Optopatch,” pipeline for high-throughput functional characteri...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: Available online 4 April 2018Source: Neuroscience LettersAuthor(s): Dao K.H. Nguyen, Ravi Thombre, Jiou WangAbstractAge-dependent neurodegenerative diseases are associated with a decline in protein quality control systems including autophagy. Amyotrophic lateral sclerosis (ALS) is a motor neuron degenerative disease of complex etiology with increasing connections to other neurodegenerative conditions such as frontotemporal dementia. Among the diverse genetic causes for ALS, a striking feature is the common connection to autophagy and its associated pathways. There is a recurring theme of protein misfoldin...
Source: Neuroscience Letters - Category: Neuroscience Source Type: research
Publication date: October 2018Source: Molecular and Cellular Neuroscience, Volume 92Author(s): Rachit Bakshi, Yuehang Xu, Kaly A. Mueller, Xiqun Chen, Eric Granucci, Sabrina Paganoni, Ghazaleh Sadri-Vakili, Michael A. SchwarzschildAbstractDominant mutations in an antioxidant enzyme superoxide dismutase-1 (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease characterized by loss of motor neurons. Oxidative stress has also been linked to many of the neurodegenerative diseases and is likely a central mechanism of motor neuron death in ALS. Astrocytes derived from mutant SOD1G93A mouse mod...
Source: Molecular and Cellular Neuroscience - Category: Neuroscience Source Type: research
Conclusions: Previous reports of ALS mortality in the United States showed similar age, sex, and race distributions but with greater age-adjusted mortality rates due to the inclusion of other diseases in the case definition. When using ICD-10 data collected prior to 2017, additional review of multiple-cause of death data is required for the accurate estimation of ALS deaths.Neuroepidemiology 2018;51:96 –103
Source: Neuroepidemiology - Category: Epidemiology Source Type: research
It is still a topic of research if specific clinical presentations of motoneuron disease (MND) patients should be classified as amyotrophic lateral sclerosis (ALS) variants, i.e. primary lateral sclerosis (PLS) with predominant upper motor neuron on the one hand and pure lower motor neuron disease (LMND) on the other hand. Objective markers for ALS are to be investigated in these patients.
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Source Type: research
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