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Flex Pharma begins Phase II trial of FLX-787 for ALS in US

Flex Pharma has begun a Phase II clinical trial (COMMEND) of FLX-787 in the US to treat patients suffering from motor neuron disease (MND) with a focus on amyotrophic lateral sclerosis (ALS).
Source: Drug Development Technology - Category: Pharmaceuticals Source Type: news

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Loss of motor units (MUs) is the primary disease process in degenerative motor neuron diseases like amyotrophic lateral sclerosis (ALS). Reinnervation can compensate for MU loss. The net result is a reduced number of MUs, and increased MU size. The assessment of the number and size of MUs will be essential to study disease progression, and to study the efficacy of treatment.
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Source Type: research
The aim of the study is to evaluate the diagnostic value of triple stimulation technique (TST) and cortical excitability studies as markers of upper motor neuron degeneration in ALS patients and to compare the extent of electrophysiological changes in abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles.
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Source Type: research
Electromyographic (EMG) abnormalities that reveal denervation and reinnervation caused by lower motor neuron degeneration do not reflect the number of motor units that determines muscle strength. Consequently, motor unit activity potential (MUAP) parameters do not reflect muscle dysfunction.
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Source Type: research
Publication date: Available online 16 August 2017 Source:Respiratory Physiology & Neurobiology Author(s): Nicole L. Nichols, Taylor A. Craig, Miles A. Tanner Amyotrophic lateral sclerosis (ALS) is a devastating disease leading to progressive motor neuron degeneration and death by ventilatory failure. In a rat model of ALS (SOD1G93A), phrenic long-term facilitation (pLTF) following acute intermittent hypoxia (AIH) is enhanced greater than expected at disease end-stage but the mechanism is unknown. We suggest that one trigger for this enhancement is motor neuron death itself. Intrapleural injections of cholera toxin B f...
Source: Respiratory Physiology and Neurobiology - Category: Respiratory Medicine Source Type: research
Authors: Zhou T, Ahmad TK, Gozda K, Truong J, Kong J, Namaka M Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease, which involves the progressive degeneration of motor neurons. ALS has long been considered a disease of the grey matter; however, pathological alterations of the white matter (WM), including axonal loss, axonal demyelination and oligodendrocyte death, have been reported in patients with ALS. The present review examined motor neuron death as the primary cause of ALS and evaluated the associated WM damage that is guided by neuronal‑glial interactions. Previous studies hav...
Source: Molecular Medicine Reports - Category: Molecular Biology Tags: Mol Med Rep Source Type: research
Abstract Amyotrophic lateral sclerosis (ALS) is the most common adult degenerative motor neuron disease. Experimental evidence indicates a direct role of transactive-response DNA-binding protein 43 (TDP-43) in the pathology of ALS and other neurodegenerative diseases. TDP-43 has been identified as a major component of cytoplasmic inclusions in patients with sporadic ALS; however, the molecular basis of the disease mechanism is not yet fully understood. Fragmentation within the second RNA recognition motif (RRM2) of TDP-43 has been observed in patient tissues and may play a role in the formation of aggregates in di...
Source: Biophysical Journal - Category: Physics Authors: Tags: Biophys J Source Type: research
In this study, we examined the non-cell autonomous role of wild type and mutant HSPB1 in an astrocyte-motor neuron co-culture model system of ALS. Astrocyte-specific overexpression of wild type HSPB1 was sufficient to attenuate SOD1(G93A) astrocyte-mediated toxicity in motor neurons, whereas, overexpression of mutHSPB1 failed to ameliorate motor neuron toxicity. Expression of a phosphomimetic HSPB1 mutant in SOD1(G93A) astrocytes also reduced toxicity to motor neurons, suggesting that phosphorylation may contribute to HSPB1 mediated-neuroprotection. These data provide evidence that astrocytic HSPB1 expression may play a ce...
Source: Experimental Neurology - Category: Neurology Authors: Tags: Exp Neurol Source Type: research
Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Publication date: Available online 2 August 2017 Source:NeuroImage: Clinical Author(s): Pilar M. Ferraro, Federica Agosta, Nilo Riva, Massimiliano Copetti, Edoardo Gioele Spinelli, Yuri Falzone, Gianni Sorarù, Giancarlo Comi, Adriano Chiò, Massimo Filippi This prospective study developed an MRI-based method for identification of individual motor neuron disease (MND) patients and test its accuracy at the individual patient level in an independent sample compared with mimic disorders. 123 patients with amyotrophic lateral sclerosis (ALS), 44 patients with predominantly upper motor neuron disease (PUMN), 20 pat...
Source: NeuroImage: Clinical - Category: Radiology Source Type: research
Amyotrophic lateral sclerosis (ALS) is primarily characterized by progressive loss of motor neurons, although there is marked phenotypic heterogeneity between cases. Typical, or "classical," ALS is associated with simultaneous upper motor neuron (UMN) and lower motor neuron (LMN) involvement at disease onset, whereas atypical forms, such as primary lateral sclerosis and progressive muscular atrophy, have early and predominant involvement in the UMN and LMN, respectively. The varying phenotypes can be so distinctive that they would seem to have differing biology. Because the same phenotypes can have multiple cause...
Source: Cold Spring Harbor perspectives in medicine - Category: Research Authors: Tags: Prion Diseases PERSPECTIVES Source Type: research
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