The Composition, Development, and Regeneration of Neuromuscular Junctions.

The Composition, Development, and Regeneration of Neuromuscular Junctions. Curr Top Dev Biol. 2018;126:99-124 Authors: Liu W, Chakkalakal JV Abstract The neuromuscular junction (NMJ) is the specialized site that connects the terminal of a motor neuron axon to skeletal muscle. As a synapse NMJ integrity is essential for transducing motor neuron signals that initiate skeletal muscle contraction. Many diseases and skeletal muscle aging are linked to impaired NMJ function and the associated muscle wasting. In this chapter we review the components of an NMJ and, the processes of NMJ development, maturation, and regeneration. Also, we briefly discuss the cellular and molecular mechanisms of NMJ decline in the context of disease and aging. PMID: 29305005 [PubMed - in process]
Source: Current Topics in Developmental Biology - Category: Biology Authors: Tags: Curr Top Dev Biol Source Type: research

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Despite being an early event in ALS, it remains unclear whether the denervation of neuromuscular junctions (NMJ) is simply the first manifestation of a globally degenerating motor neuron. Usingin vivo imaging of single axons and their NMJs over a three-month period, we identify that single motor-units are dismantled asynchronously inSOD1G37R mice. We reveal that weeks prior to complete axonal degeneration, the dismantling of axonal branches is accompanied by contemporaneous new axonal sprouting resulting in synapse formation onto nearby NMJs. Denervation events tend to propagate from the first lost NMJ, consistent with a c...
Source: eLife - Category: Biomedical Science Tags: Neuroscience Source Type: research
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Source: Neurochemical Research - Category: Neuroscience Authors: Tags: Neurochem Res Source Type: research
The mammalian imprintedDlk1-Dio3locus produces multiple long non-coding RNAs (lncRNAs) from the maternally inherited allele, includingMeg3 (i.e.,Gtl2) in the mammalian genome. Although this locus has well-characterized functions in stem cell and tumor contexts, its role during neural development is unknown. By profiling cell types at each stage of embryonic stem cell derived motor neurons (ESC~MNs) that recapitulate spinal cord development, we uncovered that lncRNAs expressed from theDlk1-Dio3locus are predominantly and gradually enriched in rostral motor neurons (MNs). Mechanistically,Meg3and otherDlk1-Dio3 locus-derived ...
Source: eLife - Category: Biomedical Science Tags: Developmental Biology Neuroscience Source Type: research
Publication date: Available online 10 October 2018Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Francesca Massenzio, Emiliano Peña-Altamira, Sabrina Petralla, Marco Virgili, Giampaolo Zuccheri, Andrea Miti, Elisabetta Polazzi, Ilaria Mengoni, Deborah Piffaretti, Barbara MontiAbstractAmyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease. Mutations in the gene encoding copper/zinc superoxide dismutase-1 (SOD1) are responsible for most familiar cases, but the role of mutant SOD1 protein dysfunction in non-cell autonomous neurodegeneration, especially in relation to mic...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - Category: Molecular Biology Source Type: research
NIH Director's Wednesday Afternoon Lecture Series The Department of Neurodegenerative Disease ’ s research portfolio encompasses prion diseases (predominantly within the embedded MRC Prion Unit), Alzheimer ’ s disease and related disorders, Huntington ’ s disease, motor neurone disease and frontotemporal dementia and studies of the pathways of cellular senescence. The research philosophy is to combine basic and clinical translational research; many of the key contributions towards understanding the basic biology of these diseases have come from clinical and neuropathological observations, and efficient tr...
Source: Videocast - All Events - Category: General Medicine Tags: Upcoming Events Source Type: video
Authors: Pinto WBVR, Naylor FGM, Chieia MAT, de Souza PVS, Oliveira ASB Abstract Facial-onset sensory and motor neuronopathy (FOSMN) syndrome represents a rare, slowly progressive, lower motor neuron disease with sensory compromise, involving mainly the face, bulbar region and upper limbs. However, non-motor symptoms and neurogenetic studies have rarely been evaluated in large case series. In the present study, 10 unrelated Brazilian patients with FOSMN syndrome underwent extensive clinical, laboratory, neurophysiological and neurogenetic assessment. Median age at symptom onset was 52.1 years, and men and women wer...
Source: Revue Neurologique - Category: Neurology Tags: Rev Neurol (Paris) Source Type: research
Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
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Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Other Mus musculus Source Type: research
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Source: Neuroscience - Category: Neuroscience Source Type: research
This article discusses the basic components of BCI for rehabilitation, including recording systems and locations, signal processing and translation algorithms, and external devices controlled through BCI commands. An overview of applications in motor and sensory restoration is provided, along with ethical questions and user perspectives regarding BCI technology.
Source: PMandR - Category: Rehabilitation Source Type: research
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