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First FDA-Approved Treatment For ALS In 22 Years Now Available In U.S.

RADICAVA™ (edaravone) Indicated for All Adult Patients Diagnosed with ALS JERSEY CITY, N.J., Aug. 8, 2017 -- (Healthcare Sales &Marketing Network) -- Mitsubishi Tanabe Pharma America, Inc., today announced RADICAVA ™ (edaravone), an intravenous therap... Biopharmaceuticals, Neurology, Product Launch Mitsubishi Tanabe Pharma, RADICAVA, edaravone, amyotrophic lateral sclerosis
Source: HSMN NewsFeed - Category: Pharmaceuticals Source Type: news

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Multisystem proteinopathy (MSP) involves disturbances of stress granule (SG) dynamics and autophagic protein degradation that underlie the pathogenesis of a spectrum of degenerative diseases that affect muscle, brain, and bone. Specifically, identical mutations in the autophagic adaptor SQSTM1 can cause varied penetrance of 4 distinct phenotypes: amyotrophic lateral sclerosis (ALS), frontotemporal dementia, Paget’s disease of the bone, and distal myopathy. It has been hypothesized that clinical pleiotropy relates to additional genetic determinants, but thus far, evidence has been lacking. Here, we provide evidence th...
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
ConclusionOur study reports retinal vessel pathology in ALS patients. These changes may be related to those observed in SBVs in skin and muscle biopsies. Furthermore, we report a thinning of the ONL in ALS, revealing a possible affection of rods and cones function in ALS.
Source: Journal of Neurology - Category: Neurology Source Type: research
Publication date: Available online 12 February 2018 Source:Neuroscience Author(s): HaoJie Hu, HuiQian Lin, WeiSong Duan, Can Cui, ZhongYao Li, YaKun Liu, Wan Wang, Di Wen, Ying Wang, ChunYan Li Amyotrophic lateral sclerosis (ALS) is a chronic, fatal neurodegenerative disorder characterized by the progressive loss of upper and lower motor neurons. Currently, there is no effective drug for ALS. Recent studies in ALS model mice have shown that insulin-like growth factor-1 (IGF1) may be a promising therapeutic drug. We demonstrate that self-complementary adeno-associated virus serum type 9 encoding the human IGF1 (scAAV9&ndas...
Source: Neuroscience - Category: Neuroscience Source Type: research
In amyotrophic lateral sclerosis (ALS) and animal models of ALS, includingSOD1-G93A mice, disassembly of the neuromuscular synapse precedes motor neuron loss and is sufficient to cause a decline in motor function that culminates in lethal respiratory paralysis. We treatedSOD1-G93A mice with an agonist antibody to MuSK, a receptor tyrosine kinase essential for maintaining neuromuscular synapses, to determine whether increasing muscle retrograde signaling would slow nerve terminal detachment from muscle. The agonist antibody, delivered after disease onset, slowed muscle denervation, promoting motor neuron survival, improving...
Source: eLife - Category: Biomedical Science Tags: Neuroscience Source Type: research
AbstractTDP-43 accumulates in nerve cells of nearly all cases of amyotrophic lateral sclerosis (ALS; the commonest form of motor neuron disease) and in the majority of Tau-negative frontotemporal lobar degeneration (FTLD). There is currently no biochemical test or marker of disease activity for ALS or FTLD, and the clinical diagnosis depends on the opinion of an experienced neurologist. TDP-43 has a key role in the pathogenesis of ALS/FTLD. Measuring TDP-43 in easily accessible biofluids, such as blood or cerebrospinal fluid, might reduce diagnostic delay and offer a readout for use in future drug trials. However, attempts...
Source: Molecular Neurobiology - Category: Neurology Source Type: research
AbstractIn the current work two novel parameters, fiber density (FD) and mean diffusion signal (MDS) are investigated for evaluating neurodegenerative processes in amyotrophic lateral sclerosis (ALS). The MDS provides a measure of the FD but is derived directly from the diffusion signal. Using tract-based spatial statistics (TBSS), pathological changes across the entire white matter and changes in the parameters over time were evaluated. The results were related to those obtained using the fractional anisotropy (FA) value. A widespread pattern of significantly decreased FD and MDS values was observed. A strong trend toward...
Source: Clinical Neuroradiology - Category: Neurology Source Type: research
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that involves both upper and lower motor neurons and eventually leads to muscle weakness, muscle atrophy, bulbar palsy, and respiratory failure. Creatine kinase (CK) is an enzyme that catalyzes the reversible conversion of creatine and utilizes adenosine triphosphate (ATP) to generate phosphocreatine and adenosine diphosphate. This enzyme is important in tissues and cells that rapidly consume ATP, particularly skeletal muscle.
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Source Type: research
Publication date: Available online 17 February 2018 Source:The Journal of Molecular Diagnostics Author(s): Claudia Cagnoli, Alessandro Brussino, Cecilia Mancini, Marina Ferrone, Laura Orsi, Paola Salmin, Patrizia Pappi, Elisa Giorgio, Elisa Pozzi, Simona Cavalieri, Eleonora Di Gregorio, Marta Ferrero, Alessandro Filla, Giuseppe De Michele, Cinzia Gellera, Caterina Mariotti, Suran Nethisinghe, Paola Giunti, Giovanni Stevanin, Alfredo Brusco Spinocerebellar ataxias (SCA) type 1, 2, 3, 6, and 7, associated with a (CAG)n repeat expansion in coding sequences, are the most prevalent autosomal dominant ataxias worldwide (approxi...
Source: The Journal of Molecular Diagnostics - Category: Pathology Source Type: research
Conclusions PAS may be considered a useful tool when investigating NMDA-mediated neocortical networks in ALS patients and the modulation of such networks after anti-glutamatergic drug intake.
Source: Brain Stimulation - Category: Neurology Source Type: research
Neuroinflammation is a devastating pathophysiological process that results in brain damage and neuronal death. Pathogens, cell fragments and cellular dysfunction trigger inflammatory responses. Irrespective of the cause, inflammasomes are key intracellular multiprotein signalling platforms that sense neuropathological conditions. The activation of inflammasomes leads to the auto‐proteolytic cleavage of caspase‐1, resulting in the proteolysis of the pro‐inflammatory cytokines interleukin (IL)1β and IL18 into their bioactive forms. It also initiates pyroptosis, a type of cell death. The two cytokines contribute to...
Source: Journal of Neuroendocrinology - Category: Endocrinology Authors: Tags: REVIEW ARTICLE Source Type: research
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