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https://www.readbyqxmd.com/read/28822818/phrenic-long-term-facilitation-following-intrapleural-ctb-sap-induced-respiratory-motor-neuron-death
#1
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 (SOD1(G93A)), 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 fragment conjugated to saporin (CTB-SAP) selectively kill respiratory motor neurons and mimic motor neuron death observed in SOD1(G93A) rats...
August 16, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28822456/-human-umbilical-cord-mesenchymal-stem-cell-transplantation-for-the-treatment-of-two-noncontinuous-segments-spinal-cord-compression-injury-in-rabbits
#2
C H Yang, B Q Yu, Q H You, J J Feng
Objective: In order to explore the effects of human umbilical cord mesenchymal stem cells (UCMSC) transplantation on the treatment of two noncontinuous segments spinal cord compression injury and to investigate whether repeated intravenous injection UCMSC was more beneficial for the recovery of spinal cord function. Methods: A total of 30 adult rabbits were randomly divided into three groups: control group (received PBS), single injection group, repeated injection group with 3 days intervals. A noncontinuous two segments SCI model was established by using the 2F Fogarty balloon catheter...
August 8, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28822104/comparison-of-the-toxic-effects-of-quinolinic-acid-and-3-nitropropionic-acid-in-c-elegans-involvement-of-the-skn-1-pathway
#3
Ilan Kotlar, Aline Colonnello, María Fernanda Aguilera-González, Daiana Silva Avila, María Eduarda de Lima, Rodolfo García-Contreras, Alma Ortíz-Plata, Félix Alexandre Antunes Soares, Michael Aschner, Abel Santamaría
The tryptophan metabolite, quinolinic acid (QUIN), and the mitochondrial toxin 3-nitropropionic acid (3-NP) are two important tools for toxicological research commonly used in neurotoxic models of excitotoxicity, oxidative stress, energy depletion, and neuronal cell death in mammals. However, their toxic properties have yet to be explored in the nematode Caenorhabditis elegans (C. elegans) for the establishment of novel, simpler, complementary, alternative, and predictive neurotoxic model of mammalian neurotoxicity...
August 18, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28822057/short-term-exposure-to-enriched-environment-in-adult-rats-restores-mk-801-induced-cognitive-deficits-and-gabaergic-interneuron-immunoreactivity-loss
#4
Ane Murueta-Goyena, Naiara Ortuzar, Pascual Gargiulo, José Vicente Lafuente, Harkaitz Bengoetxea
Perinatal injections of N-methyl-D-aspartate (NMDA) receptor antagonist in rodents emulate some cognitive impairments and neurochemical alterations, such as decreased GABAergic (gamma aminobutyric acid) interneuron immunoreactivity, also found in schizophrenia. These features are pervasive, and developing neuroprotective or neurorestorative strategies is of special interest. In this work, we aimed to investigate if a short exposure to enriched environment (EE) in early adulthood (P55-P73) was an effective strategy to improve cognitive dysfunction and to restore interneuron expression in medial prefrontal cortex (mPFC) and hippocampus (HPC)...
August 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28821666/gabaergic-interneuron-differentiation-in-the-basal-forebrain-is-mediated-through-direct-regulation-of-glutamic-acid-decarboxylase-isoforms-by-dlx-homeobox-transcription-factors
#5
Trung N Le, Qing-Ping Zhou, Inma Cobos, Shunzhen Zhang, Jamie Zagozewski, Sara Japoni, Jerry Vriend, Tracie Parkinson, Guoyan Du, John L Rubenstein, David D Eisenstat
γ-amino butyric acid (GABA) is the key inhibitory neurotransmitter in the cortex but regulation of its synthesis during forebrain development is poorly understood. In the telencephalon, members of the distal-less (Dlx) homeobox gene family are expressed in, and regulate the development of, the basal ganglia primodia from which many GABAergic neurons originate and migrate to other forebrain regions. The Dlx1/Dlx2 double knockout mice die at birth with abnormal cortical development, including loss of tangential migration of GABAergic inhibitory interneurons to the neocortex (Anderson et al...
August 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28821658/opposite-synaptic-alterations-at-the-neuromuscular-junction-in-an-als-mouse-model-when-motor-units-matter
#6
Tremblay Elsa, Martineau Éric, Robitaille Richard
Denervation of the neuromuscular junction (NMJ) precedes the loss of motor neurons (MNs) in amyotrophic lateral sclerosis (ALS). ALS is characterized by a motor unit (MU) dependent vulnerability where MNs with fast-fatigable (FF) characteristics are lost first, followed by fast-fatigue resistant (FR) and slow (S) ones. However, changes of NMJ properties as a function of MU types remain debated. We hypothesized that NMJ synaptic functions would be altered precociously in a MU specific manner, prior to structural alterations of the NMJ...
August 11, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28821650/feedback-signal-from-motoneurons-influences-a-rhythmic-pattern-generator
#7
Horacio G Rotstein, Elisa Schneider, Lidia Szczupak
Motoneurons are not mere output units of neuronal circuits that control motor behavior, but participate in pattern generation. Research on the circuit that controls the crawling motor behavior in leeches indicated that motoneurons participate as modulators of this rhythmic motor pattern. Crawling results from successive bouts of elongation and contraction of the whole leech body. In the isolated segmental ganglia dopamine can induce a rhythmic antiphasic activity of the motoneurons that control contraction (DE-3 motoneurons) and elongation (CV motoneurons)...
August 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28821644/calpain-dependent-degradation-of-nucleoporins-contributes-to-motor-neuron-death-in-a-mouse-model-of-chronic-excitotoxicity
#8
Kaori Sugiyama, Tomomi Aida, Masatoshi Nomura, Ryoichi Takayanagi, Hanns U Zeilhofer, Kohichi Tanaka
Glutamate-mediated excitotoxicity induces neuronal death by altering various intracellular signaling pathways and is implicated as a common pathogenic pathway in many neurodegenerative diseases. In the case of motor neuron disease, there is significant evidence to suggest that overactivation of AMPA receptors due to deficiencies in the expression and function of glial glutamate transporter GLT1 and GLAST plays an important role in the mechanisms of neuronal death. However, a causal role for glial glutamate transporter dysfunction in motor neuron death remains unknown...
August 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28821643/changes-in-the-excitability-of-neocortical-neurons-in-a-mouse-model-of-amyotrophic-lateral-sclerosis-are-not-specific-to-corticospinal-neurons-and-are-modulated-by-advancing-disease
#9
Juhyun Kim, Ethan G Hughes, Ashwin S Shetty, Paola Arlotta, Loyal A Goff, Dwight E Bergles, Solange P Brown
Cell-type specific changes in neuronal excitability have been proposed to contribute to the selective degeneration of corticospinal neurons in amyotrophic lateral sclerosis (ALS) and to neocortical hyperexcitability, a prominent feature of both inherited and sporadic variants of the disease, but the mechanisms underlying selective loss of specific cell types in ALS are not known. We analyzed the physiological properties of distinct classes of cortical neurons in the motor cortex of hSOD1(G93A) mice of both sexes and found that they all exhibit increases in intrinsic excitability that depend on disease stage...
August 17, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28818672/glutathione-monoethyl-ester-prevents-tdp-43-pathology-in-motor-neuronal-nsc-34%C3%A2-cells
#10
Tong Chen, Bradley J Turner, Philip M Beart, Lucy Sheehan-Hennessy, Chinasom Elekwachi, Hakan Muyderman
Oxidative stress is recognised as central in a range of neurological diseases including Amyotrophic lateral sclerosis (ALS), a disease characterised by fast progressing death of motor neurons in the brain and spinal cord. Cellular pathology includes cytosolic protein aggregates in motor neurons and glia of which potentially cytotoxic hyper-phosphorylated fragments of the Transactive response DNA Binding Protein 43 kDa (TDP-43) constitute a major component. This is closely associated with an additional loss of nuclear TDP-43 expression indicating a "loss of function" mechanism, accelerating motor neuron (MN) loss...
August 14, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28817801/a-sensitized-igf1-treatment-restores-corticospinal-axon-dependent-functions
#11
Yuanyuan Liu, Xuhua Wang, Wenlei Li, Qian Zhang, Yi Li, Zicong Zhang, Junjie Zhu, Bo Chen, Philip R Williams, Yiming Zhang, Bin Yu, Xiaosong Gu, Zhigang He
A major hurdle for functional recovery after both spinal cord injury and cortical stroke is the limited regrowth of the axons in the corticospinal tract (CST) that originate in the motor cortex and innervate the spinal cord. Despite recent advances in engaging the intrinsic mechanisms that control CST regrowth, it remains to be tested whether such methods can promote functional recovery in translatable settings. Here we show that post-lesional AAV-assisted co-expression of two soluble proteins, namely insulin-like growth factor 1 (IGF1) and osteopontin (OPN), in cortical neurons leads to robust CST regrowth and the recovery of CST-dependent behavioral performance after both T10 lateral spinal hemisection and a unilateral cortical stroke...
August 16, 2017: Neuron
https://www.readbyqxmd.com/read/28817498/an-interprofessional-approach-to-amyotrophic-lateral-sclerosis-care
#12
Jerri L Jaffa, Marlene Dufault, Mary Lavin
Amyotrophic lateral sclerosis (ALS) is a progressive fatal neurodegenerative disease that is characterized by the death of motor neurons in the spinal cord, brain stem, and motor cortex that are responsible for voluntary movement. For unknown reasons, military veterans are approximately twice as likely as the public to be given a diagnosis of ALS. Currently, there is no cure for ALS. The only US Food and Drug Administration-approved disease-modifying medication is riluzole (Rilutek), which may slow ALS progression but only minimally increases survival time...
August 16, 2017: Journal of Neuroscience Nursing: Journal of the American Association of Neuroscience Nurses
https://www.readbyqxmd.com/read/28817497/self-care-in-people-with-motor-neuron-disease-an-integrative-review
#13
Barbara Bassola, Maura Lusignani
INTRODUCTION: Self-care is a crucial aspect in the management of chronic diseases. People with motor neuron disease (MND) live with a chronic degenerative condition in rapid evolution, which leads them to high dependencies. During their illness, they must apply several behaviors including adherence to ventilation and nutritional therapy. OBJECTIVE: The purpose of this review is to systematically explore the concept of self-care for people with MND. METHOD: An integrative review of the literature has been conducted...
August 16, 2017: Journal of Neuroscience Nursing: Journal of the American Association of Neuroscience Nurses
https://www.readbyqxmd.com/read/28817209/huntington-s-disease-a-clinical-review
#14
Peter McColgan, Sarah J Tabrizi
Huntington's disease (HD) is a fully penetrant neurodegenerative disease caused by a dominantly inherited CAG trinucleotide repeat expansion in the huntingtin gene on chromosome 4. In Western populations HD has a prevalence of 10.6-13.7 individuals per 100,000. It is characterised by cognitive, motor and psychiatric disturbance. At the cellular level mutant huntingtin results in neuronal dysfunction and death through a number of mechanisms, including disruption of proteostasis, transcription and mitochondrial function and direct toxicity of the mutant protein...
August 17, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28816645/a-systems-model-of-parkinson-s-disease-using-biochemical-systems-theory
#15
Hemalatha Sasidharakurup, Nidheesh Melethadathil, Bipin Nair, Shyam Diwakar
Parkinson's disease (PD), a neurodegenerative disorder, affects millions of people and has gained attention because of its clinical roles affecting behaviors related to motor and nonmotor symptoms. Although studies on PD from various aspects are becoming popular, few rely on predictive systems modeling approaches. Using Biochemical Systems Theory (BST), this article attempts to model and characterize dopaminergic cell death and understand pathophysiology of progression of PD. PD pathways were modeled using stochastic differential equations incorporating law of mass action, and initial concentrations for the modeled proteins were obtained from literature...
August 2017: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/28815019/therapeutic-potential-of-systemic-brain-rejuvenation-strategies-for-neurodegenerative-disease
#16
REVIEW
Alana M Horowitz, Saul A Villeda
Neurodegenerative diseases are a devastating group of conditions that cause progressive loss of neuronal integrity, affecting cognitive and motor functioning in an ever-increasing number of older individuals. Attempts to slow neurodegenerative disease advancement have met with little success in the clinic; however, a new therapeutic approach may stem from classic interventions, such as caloric restriction, exercise, and parabiosis. For decades, researchers have reported that these systemic-level manipulations can promote major functional changes that extend organismal lifespan and healthspan...
2017: F1000Research
https://www.readbyqxmd.com/read/28814634/state-dependent-sensorimotor-gating-in-a-rhythmic-motor-system
#17
Rachel S White, Robert M Spencer, Michael P Nusbaum, Dawn M Blitz
Sensory feedback influences motor circuits and/or their projection neuron inputs to adjust ongoing motor activity, but its efficacy varies. Currently, less is known about regulation of sensory feedback onto projection neurons that control downstream motor circuits than about sensory regulation of the motor circuit neurons themselves. Here, we tested whether sensory feedback onto projection neurons is sensitive only to activation of a motor system, or also to the modulatory state underlying that activation, using the crab Cancer borealis stomatogastric nervous system...
August 16, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28814041/assessing-bradykinesia-in-parkinson-s-disease-using-gyroscope-signals
#18
S Summa, J Tosi, F Taffoni, L Di Biase, M Marano, A Cascio Rizzo, M Tombini, G Di Pino, D Formica
Parkinson's disease (PD) is a neurodegenerative brain disorder that slowly brings on the dopaminergic neurons death. The depletion of the dopaminergic signal causes the onset of motor symptoms such as tremor, bradykinesia and rigidity. Usually, neurologists regularly monitor motor symptoms and motor fluctuations using the MDS-UPDRS part III clinical scale. Nevertheless, to have a more objective and quantitative evaluation, it is possible to assess the cardinal motor symptoms of PD using wearable sensors and portable robotic devices...
July 2017: IEEE ... International Conference on Rehabilitation Robotics: [proceedings]
https://www.readbyqxmd.com/read/28813912/transverse-forces-versus-modified-ashworth-scale-for-upper-limb-flexion-extension-in-para-sagittal-plane
#19
Nitin Seth, Denise Johnson, Hussein A Abdullah
Spasticity is a common impairment following an upper motor neuron lesion in conditions such as stroke and brain injury. A clinical issue is how to best quantify and measure spasticity. Recently, research has been performed to develop new methods of spasticity quantification using various systems. This paper follows up on previous work taking a closer look at the role of transversal forces obtained via rehabilitation robot for motions in the para-sagittal plane. Results from 45 healthy individuals and 40 individuals with acquired brain injury demonstrate that although the passive upper motions are vertical, horizontal forces into and away from the individual's body demonstrate a relationship with the Modified Ashworth Scale...
July 2017: IEEE ... International Conference on Rehabilitation Robotics: [proceedings]
https://www.readbyqxmd.com/read/28812939/removing-dysfunctional-mitochondria-from-axons-independent-of-mitophagy-under-pathophysiological-conditions
#20
Mei-Yao Lin, Xiu-Tang Cheng, Yuxiang Xie, Qian Cai, Zu-Hang Sheng
Chronic mitochondrial dysfunction has been implicated in major neurodegenerative diseases. Long-term cumulative pathological stress leads to axonal accumulation of damaged mitochondria. Therefore, the early removal of defective mitochondria from axons constitutes a critical step of mitochondrial quality control. We recently investigated the axonal mitochondrial response to mild stress in wild-type neurons and chronic mitochondrial defects in amyotrophic lateral sclerosis (ALS)- and Alzheimer disease (AD)-linked neurons...
August 16, 2017: Autophagy
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