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https://www.readbyqxmd.com/read/28108397/aav9-igf1-protects-tdp-25-cells-from-apoptosis-and-oxidative-stress-partly-via-up-regulating-the-expression-of-vegf-in-vitro
#1
Zhongyao Li, Weisong Duan, Can Cui, Yaling Liu, Chunyan Li, Yakun Liu
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease lacking of curable treatments to date. Adeno-associated virus (AAV) vectors make gene therapy an effective strategy in treating neurological disorders. Despite Kaspar and colleagues have showed that AAV-IGF1 delivery successfully prolonged the survival of SOD1G93A mice, whether IGF-1 act as a protective role in the TDP-43 mutant model still have not been reported. In this study, we proved that AAV9 vector mediated expression of human wild-type IGF-1 protected TDP-25 cells from apoptosis and oxidative stress...
January 17, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28102472/erratum-to-administration-of-17%C3%AE-estradiol-improves-motoneuron-survival-and-down-regulates-inflammasome-activation-in-male-sod1-g93a-als-mice
#2
Marius Heitzer, Sarah Kaiser, Mithila Kanagaratnam, Adib Zendedel, Philipp Hartmann, Cordian Beyer, Sonja Johann
No abstract text is available yet for this article.
January 19, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28102336/differential-effects-of-phytotherapic-preparations-in-the-hsod1-drosophila-melanogaster-model-of-als
#3
Francescaelena De Rose, Roberto Marotta, Giuseppe Talani, Tiziano Catelani, Paolo Solari, Simone Poddighe, Giuseppe Borghero, Francesco Marrosu, Enrico Sanna, Sanjay Kasture, Elio Acquas, Anna Liscia
The present study was aimed at characterizing the effects of Withania somnifera (Wse) and Mucuna pruriens (Mpe) on a Drosophila melanogaster model for Amyotrophic Lateral Sclerosis (ALS). In particular, the effects of Wse and Mpe were assessed following feeding the flies selectively overexpressing the wild human copper, zinc-superoxide dismutase (hSOD1-gain-of-function) in Drosophila motoneurons. Although ALS-hSOD1 mutants showed no impairment in life span, with respect to GAL4 controls, the results revealed impairment of climbing behaviour, muscle electrophysiological parameters (latency and amplitude of ePSPs) as well as thoracic ganglia mitochondrial functions...
January 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28101533/experimental-design-and-data-analysis-issues-contribute-to-inconsistent-results-of-c-bouton-changes-in-amyotrophic-lateral-sclerosis
#4
S Shekar Dukkipati, Aouatef Chihi, Yiwen Wang, Sherif M Elbasiouny
The possible presence of pathological changes in cholinergic synaptic inputs [cholinergic boutons (C-boutons)] is a contentious topic within the ALS field. Conflicting data reported on this issue makes it difficult to assess the roles of these synaptic inputs in ALS. Our objective was to determine whether the reported changes are truly statistically and biologically significant and why replication is problematic. This is an urgent question, as C-boutons are an important regulator of spinal motoneuron excitability, and pathological changes in motoneuron excitability are present throughout disease progression...
January 2017: ENeuro
https://www.readbyqxmd.com/read/28100513/loss-of-ranbp2-in-motor-neurons-causes-the-disruption-of-nucleocytoplasmic-and-chemokine-signaling-and-proteostasis-of-hnrnph3-and-mmp28-and-the-development-of-amyotrophic-lateral-sclerosis-als-like-syndromes
#5
Kyoung-In Cho, Dosuk Yoon, Sunny Qiu, Zachary Danziger, Warren M Grill, William C Wetsel, Paulo A Ferreira
The pathogenic drivers of sporadic and familial motor neuron disease (MND), such ALS, are unknown. MND impair the Ran GTPase cycle, which controls nucleocytoplasmic transport, ribostasis and proteostasis; however, cause-effect mechanisms of Ran GTPase modulators in motoneuron pathobiology are heretofore elusive. The cytosolic and peripheral nucleoporin, Ranbp2, is a critical regulator of the Ran GTPase cycle and proteostasis of neurological disease-prone substrates, but the roles of Ranbp2 in motoneuron biology and disease remain unknown...
January 18, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28097486/the-therapeutic-effectiveness-of-delayed-fetal-spinal-cord-tissue-transplantation-on-respiratory-function-following-mid-cervical-spinal-cord-injury
#6
Chia-Ching Lin, Sih-Rong Lai, Yu-Han Shao, Chun-Lin Chen, Kun-Ze Lee
Respiratory impairment due to damage of the spinal respiratory motoneurons and interruption of the descending drives from brainstem premotor neurons to spinal respiratory motoneurons is the leading cause of morbidity and mortality following cervical spinal cord injury. The present study was designed to evaluate the therapeutic effectiveness of delayed transplantation of fetal spinal cord (FSC) tissue on respiratory function in rats with mid-cervical spinal cord injury. Embryonic day-14 rat FSC tissue was transplanted into a C4 spinal cord hemilesion cavity in adult male rats at 1 week postinjury...
January 17, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28095293/mapk-signaling-promotes-axonal-degeneration-by-speeding-the-turnover-of-the-axonal-maintenance-factor-nmnat2
#7
Lauren J Walker, Daniel W Summers, Yo Sasaki, E J Brace, Jeffrey Milbrandt, Aaron DiAntonio
Injury-induced (Wallerian) axonal degeneration is regulated via the opposing actions of pro-degenerative factors such as SARM1 and a MAPK signal and pro-survival factors, the most important of which is the NAD(+) biosynthetic enzyme NMNAT2 that inhibits activation of the SARM1 pathway. Here we investigate the mechanism by which MAPK signaling facilitates axonal degeneration. We show that MAPK signaling promotes the turnover of the axonal survival factor NMNAT2 in cultured mammalian neurons as well as the Drosophila ortholog dNMNAT in motoneurons...
January 17, 2017: ELife
https://www.readbyqxmd.com/read/28091925/changes-in-the-neurochemical-composition-of-motor-neurons-of-the-spinal-cord-in-mice-under-conditions-of-space-flight
#8
V V Porseva, V V Shilkin, A A Strelkov, I B Krasnov, P M Masliukov
Expression of choline acetyltransferase, 200-kDa neurofilament protein, 28-kDa calbindin, neuronal NO synthase, caspase 3, and Ki-67 in the motor neurons of spinal cord segments T3-T5 in male C57Bl/6 mice after 30-day space flight in the Bion-M1 biosatellite was studied by immunohistochemical methods. Under conditions space flight, the size of motoneurons increased, the number of neurons containing choline acetyltransferase and neurofilaments, decreased, and the number of calbindin-positive neurons increased; motoneurons, expressing neuronal NO synthase and caspase 3 appeared, while Ki-67 was not detected...
January 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28078066/use-it-or-lose-it-tonic-activity-of-slow-motoneurons-promotes-their-survival-and-preferentially-increases-slow-fiber-type-groupings-in-muscles-of-old-lifelong-recreational-sportsmen
#9
Simone Mosole, Ugo Carraro, Helmut Kern, Stefan Loefler, Sandra Zampieri
: Histochemistry, immuno-histochemistry, gel electrophoresis of single muscle fibers and electromyography of aging muscles and nerves suggest that: i) denervation contributes to muscle atrophy, ii) impaired mobility accelerates the process, and iii) lifelong running protects against loss of motor units. Recent corroborating results on the muscle effects of Functional Electrical Stimulation (FES) of aged muscles will be also mentioned, but we will in particular discuss how and why a lifelong increased physical activity sustains reinnervation of muscle fibers...
September 15, 2016: European Journal of Translational Myology
https://www.readbyqxmd.com/read/28059804/bupivacaine-increases-the-rate-of%C3%A2-motoneuron-death-following-peripheral%C3%A2-nerve-injury
#10
Susanna C Byram, Scott W Byram, Nicholas M Miller, Keith N Fargo
BACKGROUND: Appropriate management of pain after an injury or surgical procedure has been shown to improve patient outcomes. While infrequent, nerve damage resulting from regional anesthesia can be devastating, however the mechanism remains unknown. Local anesthetics are neurotoxic yet are frequently applied to sites where peripheral nerves are regenerating. Therefore, understanding their effects on injured and growing neurons may have important implications for clinical practice. OBJECTIVE: The purpose of this study was to determine if local anesthetics exacerbate the rate of motoneuron death following axotomy...
January 4, 2017: Restorative Neurology and Neuroscience
https://www.readbyqxmd.com/read/28053245/synaptic-control-of-the-shape-of-the-motoneuron-pool-input-output-function
#11
Randall K Powers, Charles J Heckman
Although motoneurons have often been considered to be fairly linear transducers of synaptic input, recent evidence suggests that strong persistent inward currents (PICs) in motoneurons allow neuromodulatory and inhibitory synaptic inputs to induce large nonlinearities in the relation between the level of excitatory input and motor output. To try and estimate the possible extent of this nonlinearity, we developed a pool of model motoneurons designed to replicate the characteristics of motoneuron input-output properties measured in medial gastrocnemius motoneurons in the decerebrate cat using voltage-clamp and current-clamp techniques...
January 4, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28053029/mutant-%C3%AE-synuclein-overexpression-induces-stressless-pacemaking-in-vagal-motoneurons-at-risk-in-parkinson-s-disease
#12
Efrat Lasser-Katz, Alon Simchovitz, Wei-Hua Chiu, Wolfgang H Oertel, Ronit Sharon, Hermona Soreq, Jochen Roeper, Joshua A Goldberg
: α-Synuclein overexpression (ASOX) drives the formation of toxic aggregates in neurons vulnerable in Parkinson's disease (PD), including dopaminergic neurons of the substantia nigra (SN) and cholinergic neurons of the dorsal motor nucleus of the vagus (DMV). Just as these populations differ in when they exhibit α-synucleinopathies during PD pathogenesis, they could also differ in their physiological responses to ASOX. An ASOX-mediated hyperactivity of SN dopamine neurons, which was caused by oxidative dysfunction of Kv4...
January 4, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28051949/stereological-assessment-of-the-total-number-of-hypoglossal-neurons-after-repeated-crush-injuries-to-the-hypoglossal-nerve-in-adult-rats
#13
Nanae Fukushima, Mika Karasawa, Kumiko Yokouchi, Norimi Sumitomo, Kyutaro Kawagishi, Tetsuji Moriizumi
OBJECTIVE: Retrograde neuronal cell death does not occur in mature motoneurons following the axonal injury of peripheral nerves. However, a previous study suggested that retrograde neuronal cell death does occur in adult rats after the creation of double lesions on the hypoglossal (XII) nerve based on a substantial decrease in the number of XII neurons. Using stereological methods, we examined neuronal apoptosis in XII neurons and the total number of XII neurons following repeated crush injuries to the XII nerve...
February 2017: Neurological Research
https://www.readbyqxmd.com/read/28049844/contribution-of-propriospinal-neurons-to-recovery-of-hand-dexterity-after-corticospinal-tract-lesions-in-monkeys
#14
Takamichi Tohyama, Masaharu Kinoshita, Kenta Kobayashi, Kaoru Isa, Dai Watanabe, Kazuto Kobayashi, Meigen Liu, Tadashi Isa
The direct cortico-motoneuronal connection is believed to be essential for the control of dexterous hand movements, such as precision grip in primates. It was reported, however, that even after lesion of the corticospinal tract (CST) at the C4-C5 segment, precision grip largely recovered within 1-3 mo, suggesting that the recovery depends on transmission through intercalated neurons rostral to the lesion, such as the propriospinal neurons (PNs) in the midcervical segments. To obtain direct evidence for the contribution of PNs to recovery after CST lesion, we applied a pathway-selective and reversible blocking method using double viral vectors to the PNs in six monkeys after CST lesions at C4-C5...
January 17, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28048973/effect-of-physical-exercise-and-anabolic-steroid-treatment-on-spinal-motoneurons-and-surrounding-glia-of-wild-type-and-als-mice
#15
Roman M Kassa, Roberta Bonafede, Federico Boschi, Marina Bentivoglio, Raffaella Mariotti
Motoneuron degeneration is the hallmark of amyotrophic lateral sclerosis (ALS). The cause and predisposing factors for sporadic ALS are still unknown. Exposure to a specific environmental risk factors in subjects with a susceptibility genotype may increase the risk of the disease. The role of physical activity and the use of anabolic steroids are still debated in epidemiological studies on patients and murine models of ALS. To assess at the cellular level the role (beneficial or detrimental) of physical exercise and the use of anabolic steroid, we here investigated, in SOD1(G93A) (mSOD1) mice and wild-type littermates, changes in the ventral horn after regular exercise, treatment with the anabolic androgenic steroid 19-nortestosterone (nandrolone), and their combination, compared with matched control sedentary mice...
December 31, 2016: Brain Research
https://www.readbyqxmd.com/read/28045911/do-quiescence-and-wasp-venom-induced-lethargy-share-common-neuronal-mechanisms-in-cockroaches
#16
Stav Emanuel, Frederic Libersat
The escape behavior of a cockroach may not occur when it is either in a quiescent state or after being stung by the jewel wasp (Ampulex compressa). In the present paper, we show that quiescence is an innate lethargic state during which the cockroach is less responsive to external stimuli. The neuronal mechanism of such a state is poorly understood. In contrast to quiescence, the venom-induced lethargic state is not an innate state in cockroaches. The Jewel Wasp disables the escape behavior of cockroaches by injecting its venom directly in the head ganglia, inside a neuropile called the central complex a 'higher center' known to regulate motor behaviors...
2017: PloS One
https://www.readbyqxmd.com/read/28039767/static-otolithic-drive-alters-presynaptic-inhibition-in-soleus-motor-pool
#17
Apollonia Fox, David Koceja
The vestibular system has both direct and indirect connections to the soleus motor pool via the vestibulospinal and reticulospinal tracts. The exact nature of how this vestibular information is integrated within the spinal cord is largely unknown. The purpose of this study was to identify whether changes in static otolithic drive altered the amount of presynaptic inhibition in the soleus H-reflex pathway. Changes in static otolithic drive were investigated in sixteen healthy participants using a tilt table...
December 20, 2016: Journal of Electromyography and Kinesiology
https://www.readbyqxmd.com/read/28039540/f-wave-of-single-firing-motor-units-correct-or-misleading-criterion-of-motoneuron-excitability-in-humans
#18
Lydia P Kudina, Regina E Andreeva
Motoneuron excitability is a critical property for information processing during motor control. F-wave (a motoneuronal recurrent discharge evoked by a motor antidromic volley) is often used as a criterion of motoneuron pool excitability in normal and neuromuscular diseases. However, such using of F-wave calls in question. The present study was designed to explore excitability of single low-threshold motoneurons during their natural firing in healthy humans and to ascertain whether F-wave is a correct measure of motoneuronal excitability...
December 31, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/28036084/preferential-enhancement-of-sensory-and-motor-axon-regeneration-by-combining-extracellular-matrix-components-with-neurotrophic-factors
#19
Daniel Santos, Francisco González-Pérez, Guido Giudetti, Silvestro Micera, Esther Udina, Jaume Del Valle, Xavier Navarro
After peripheral nerve injury, motor and sensory axons are able to regenerate but inaccuracy of target reinnervation leads to poor functional recovery. Extracellular matrix (ECM) components and neurotrophic factors (NTFs) exert their effect on different neuronal populations creating a suitable environment to promote axonal growth. Here, we assessed in vitro and in vivo the selective effects of combining different ECM components with NTFs on motor and sensory axons regeneration and target reinnervation. Organotypic cultures with collagen, laminin and nerve growth factor (NGF)/neurotrophin-3 (NT3) or collagen, fibronectin and brain-derived neurotrophic factor (BDNF) selectively enhanced sensory neurite outgrowth of DRG neurons and motor neurite outgrowth from spinal cord slices respectively...
December 29, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28033684/cellular-reactions-and-compensatory-tissue-re-organization-during-spontaneous-recovery-after-spinal-cord-injury-in-neonatal-mice
#20
Rishab S Chawla, Mark Züchner, Maria Mastrangelopoulou, François M Lambert, Joel C Glover, Jean-Luc Boulland
Following incomplete spinal cord injuries, neonatal mammals display a remarkable degree of behavioral recovery. Previously, we have demonstrated in neonatal mice a wholesale re-establishment and reorganization of synaptic connections from some descending axon tracts (Boulland et al., 2013). To assess the potential cellular mechanisms contributing to this recovery, we have here characterized a variety of cellular sequelae following thoracic compression injuries, focusing particularly on cell loss and proliferation, inflammation and reactive gliosis, and the dynamics of specific types of synaptic terminals...
December 29, 2016: Developmental Neurobiology
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