Organs-on-chip technology reveals new drug candidates for Lou Gehrig's disease

The investigation of amyotrophic lateral sclerosis (ALS) - also known as Lou Gehrig's disease - through muscle-on-a-chip technology has revealed a new drug combination that may serve as an effective treatment of the progressive neurodegenerative disease. These findings highlight organ-on-a-chip technologies - in which live conditions of the body are mimicked in a microfluidic cell culture - as promising platforms for
Source: World Pharma News - Category: Pharmaceuticals Tags: Featured Research Research and Development Source Type: news

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Corrigendum to "Driven to decay: Excitability and synaptic abnormalities in amyotrophic lateral sclerosis" [Brain Research Bulletin 140 (2018) 318-333]. Brain Res Bull. 2019 Mar;146:327 Authors: Fogarty MJ PMID: 30770039 [PubMed - in process]
Source: Brain Research Bulletin - Category: Neurology Authors: Tags: Brain Res Bull Source Type: research
The FDA this week sent a warning letter to a Beverly Hills, Calif.-based surgeon for their marketing of an unapproved implantable device, dubbed the Pocket Protector, which the surgeon claims can prevent and treat capsular contracture, or scar tissue tightening, during breast implant procedures. In its letter, the federal watchdog also accuses the surgeon, Dr. Mark Berman, of “significant deviations from the FDA’s quality system requirements and current good manufacturing practices,” including charges related to the sterility of implantable devices. “This is not the first time the FDA has notified D...
Source: Mass Device - Category: Medical Devices Authors: Tags: Cosmetic/Aesthetic Featured Food & Drug Administration (FDA) Stem Cells Source Type: news
AbstractThe authors set out to study the role of T1-weighted muscle MRI in the diagnostic phase of ALS, comparing images from ten patients and nine age-matched healthy controls (HCs). All subjects underwent MRI of 68 muscles in the hands, paraspinal regions and lower limbs; the images were semi-quantitatively scored. Atrophy was more frequent in muscles of ALS patients than HCs (p 
Source: Skeletal Radiology - Category: Radiology Source Type: research
Abstract Cellular adaption to nutrient stress is exquisitely regulated, and its dysregulation could underlie human diseases including neurodegeneration. C9orf72 is linked to the most common forms of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) as well as rare cases of other neurological disorders. Recent studies have implicated C9orf72 functions in the autophagy-lysosome pathway, but the exact roles of C9orf72 remain unclear. We found that C9orf72 is required for the lysosomal targeting and degradation of CARM1, which is an important epigenetic regulator of macroautophagy/autophagy and lip...
Source: Autophagy - Category: Cytology Authors: Tags: Autophagy Source Type: research
A G4C2 hexanucleotide repeat expansion in the noncoding region of C9orf72 is the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (c9FTD/ALS). Putative disease mechanisms underlyin...
Source: Molecular Neurodegeneration - Category: Neurology Authors: Tags: Research article Source Type: research
Amyotrophic lateral sclerosis (ALS) patients show progressive respiratory muscle weakness leading to death from respiratory failure. However, there are no data on diaphragm histological changes in ALS patients and how they correlate with routine respiratory measurements. We collected 39 diaphragm biopsies concomitantly with laparoscopic insertion of intradiaphragmatic electrodes during a randomised controlled trial evaluating early diaphragm pacing in ALS (; NCT01583088). Myofibre type, size and distribution were evaluated by immunofluorescence microscopy and correlated with spirometry, respirator...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Respiratory clinical practice Original Articles: Lung structure and function Source Type: research
How hexanucleotide GGGGCC (G4C2) repeat expansions in C9orf72 cause frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is not understood. We developed a mouse model engineered to express poly(PR), a proline-arginine (PR) dipeptide repeat protein synthesized from expanded G4C2 repeats. The expression of green fluorescent protein–conjugated (PR)50 (a 50-repeat PR protein) throughout the mouse brain yielded progressive brain atrophy, neuron loss, loss of poly(PR)-positive cells, and gliosis, culminating in motor and memory impairments. We found that poly(PR) bound DNA, localized to heterochromatin, an...
Source: ScienceNOW - Category: Science Authors: Tags: Medicine, Diseases, Online Only r-articles Source Type: news
In a recentstudy inPLOS One, a 63-year-old man with tetraplegia caused by a spinal cord injury sent his first text messages with an off-the-shelf consumer tablet paired to an intracortical brain-computer interface (iBCI). He and 2 other participants with limited arm and hand mobility due to amyotrophic lateral sclerosis also used the iBCI to browse the internet, send emails, chat with researchers, stream music, watch videos, check the weather, and read the news —all by simply thinking about the tasks.
Source: JAMA - Category: General Medicine Source Type: research
otenza RL Abstract Amyotrophic lateral sclerosis (ALS) is a fatal progressing neurodegenerative disease; to date, despite the intense research effort, only two therapeutic options, with very limited effects, are available. The purinergic system has been indicated as a possible new therapeutic target for ALS, but the results are often contradictory and generally confused. The present study was designed to determine whether P1 adenosine receptor ligands affected disease progression in a transgenic model of ALS. SOD1G93A mice were chronically treated, from presymptomatic stage, with a selective adenosine A2A receptor...
Source: Neurochemical Research - Category: Neuroscience Authors: Tags: Neurochem Res Source Type: research
Mutations in coding and non-coding regions of FUS cause amyotrophic lateral sclerosis (ALS). The latter mutations may exert toxicity by increasing FUS accumulation. We show here that broad expression within the nervous system of wild-type or either of two ALS-linked mutants of human FUS in mice produces progressive motor phenotypes accompanied by characteristic ALS-like pathology. FUS levels are autoregulated by a mechanism in which human FUS downregulates endogenous FUS at mRNA and protein levels. Increasing wild-type human FUS expression achieved by saturating this autoregulatory mechanism produces a rapidly progressive ...
Source: eLife - Category: Biomedical Science Tags: Neuroscience Source Type: research
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