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

Related Links:

Publication date: Available online 18 June 2018 Source:Molecular and Cellular Neuroscience Author(s): Rachit Bakshi, Yuehang Xu, Kaly A. Mueller, Xiqun Chen, Eric Granucci, Sabrina Paganoni, Ghazaleh Sadri-Vakili, Michael A. Schwarzschild Dominant 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 SOD1 G93A mouse m...
Source: Molecular and Cellular Neuroscience - Category: Neuroscience Source Type: research
Publication date: Available online 19 June 2018 Source:The Lancet Neurology Author(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 Heneka, Siw Wollebæk Johannesen, Bettina Göricke Background Rasagiline, a monoamine oxidase B inhibitor with neuroprotective potential in Parkinson's disease, has shown a disease-modifying effect in the SOD...
Source: The Lancet Neurology - Category: Neurology Source Type: research
This study was conducted to elucidate the genetic etiology of patients with the pure type AD-HSP diagnosis. The patient group consisted of 23 individuals from 6 families in Turkey. In the first step of work, Sanger sequencing (SS) was performed inATL1,SPAST, andREEP1 genes and the second phase whole-exome sequencing (WES) was performed following SS analysis for the patients with no detected mutations in these genes. The results of this study revealed that inATL1, 6 patients have previously reported c.776C  >  A mutation and 6 patients have novel c.470 T >  C mutation. InSPAST, 3 patie...
Source: Neurological Sciences - Category: Neurology Source Type: research
Conclusions: Empathy develops and operates within shared experiences and connections, enabled by structural possibilities provided by the forums giving users the opportunity and means to interact within public, restricted, and more private spaces, as well as within groups and in one-to-one exchanges. The atmosphere and feeling of both sites and perceived audiences were important facilitators of empathy, with users sharing a perception of virtual communities of caring and supportive people. Our findings are of value to organizations hosting health forums and to health professionals signposting patients to additional sources of support.
Source: Journal of Medical Internet Research - Category: General Medicine Authors: Source Type: research
ò RS Abstract BACKGROUND: Amyotrophic Lateral Sclerosis flail arm (ALS-FA) is a motor neuron disease form confined to the upper limbs (cervical spinal cord region), often with asymmetric onset. To date, there is no defined neurorehabilitative strategy for ALS patients, although aerobic exercises may be of some help. CASE REPORT: A 69 year-old woman affected by ALS-FA was admitted to our research institute because of upper limb muscles weakness. She was then submitted to two different conventional physiotherapy programs, the first stand-alone and the second combined to a robotic treatment. The patient g...
Source: European Journal of Physical and Rehabilitation Medicine - Category: Rehabilitation Authors: Tags: Eur J Phys Rehabil Med Source Type: research
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
Spinal muscular atrophy (SMA) results from insufficient levels of the survival motor neuron (SMN) protein. Drosophila is conducive to large-scale genetic-modifier screens which can reveal novel pathways underpinn...
Source: BMC Research Notes - Category: Research Authors: Tags: Research note Source Type: research
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. PMID: 29898446 [PubMed - as supplied by publisher]
Source: Neuro-Degenerative Diseases - Category: Neurology Authors: Tags: Neurodegener Dis Source Type: research
Spinal muscular atrophy (SMA), a degenerative motor neuron (MN) disease, caused by loss of functional survival of motor neuron (SMN) protein due to SMN1 gene mutations, is a leading cause of infant mortality. Increasing SMN levels ameliorates the disease phenotype and is unanimously accepted as a therapeutic approach for patients with SMA. The ubiquitin/proteasome system is known to regulate SMN protein levels; however, whether autophagy controls SMN levels remains poorly explored. Here, we show that SMN protein is degraded by autophagy. Pharmacological and genetic inhibition of autophagy increases SMN levels, while induct...
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
CONCLUSIONS: The MND-FTD patients frequently displayed a distinctive motor pattern characterized by weakness and atrophy in distal upper limb muscles and dysphagia, with no or little spreading to other regions. These features may help to define specific subgroups of patients, which is important with regard to clinical management, outcome, and research. PMID: 29886477 [PubMed - as supplied by publisher]
Source: Dementia and Geriatric Cognitive Disorders - Category: Psychiatry Tags: Dement Geriatr Cogn Disord Source Type: research
More News: Biology | Brain | Molecular Biology | Motor Neurone Disease | Neurology