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Motor neuron disease

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https://www.readbyqxmd.com/read/29163226/adenosine-a2a-receptor-modulates-the-activity-of-globus-pallidus-neurons-in-rats
#1
Hui-Ling Diao, Yan Xue, Xiao-Hua Han, Shuang-Yan Wang, Cui Liu, Wen-Fang Chen, Lei Chen
The globus pallidus is a central nucleus in the basal ganglia motor control circuit. Morphological studies have revealed the expression of adenosine A2A receptors in the globus pallidus. To determine the modulation of adenosine A2A receptors on the activity of pallidal neurons in both normal and parkinsonian rats, in vivo electrophysiological and behavioral tests were performed in the present study. The extracellular single unit recordings showed that micro-pressure administration of adenosine A2A receptor agonist, CGS21680, regulated the pallidal firing activity...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29163149/gyy4137-an-h2s-slow-releasing-donor-prevents-nitrative-stress-and-%C3%AE-synuclein-nitration-in-an-mptp-mouse-model-of-parkinson-s-disease
#2
Xiaoou Hou, Yuqing Yuan, Yulan Sheng, Baoshi Yuan, Yali Wang, Jiyue Zheng, Chun-Feng Liu, Xiaohu Zhang, Li-Fang Hu
The neuromodulator hydrogen sulfide (H2S) was shown to exert neuroprotection in different models of Parkinson's disease (PD) via its anti-inflammatory and anti-apoptotic properties. In this study, we evaluated the effect of an H2S slow-releasing compound GYY4137 (GYY) on a mouse PD model induced by acute injection with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). GYY was intraperitoneally (i.p.) injected once daily into male C57BL/6J mice 3 days before and 2 weeks after MPTP (14 mg/kg, four times at 2-h intervals, i...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29163139/exercise-induced-neuroprotection-of-the-nigrostriatal-dopamine-system-in-parkinson-s-disease
#3
REVIEW
Lijuan Hou, Wei Chen, Xiaoli Liu, Decai Qiao, Fu-Ming Zhou
Epidemiological studies indicate that physical activity and exercise may reduce the risk of developing Parkinson's disease (PD), and clinical observations suggest that physical exercise can reduce the motor symptoms in PD patients. In experimental animals, a profound observation is that exercise of appropriate timing, duration, and intensity can reduce toxin-induced lesion of the nigrostriatal dopamine (DA) system in animal PD models, although negative results have also been reported, potentially due to inappropriate timing and intensity of the exercise regimen...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29163034/direct-reprogramming-rather-than-ipsc-based-reprogramming-maintains-aging-hallmarks-in-human-motor-neurons
#4
Yu Tang, Meng-Lu Liu, Tong Zang, Chun-Li Zhang
In vitro generation of motor neurons (MNs) is a promising approach for modeling motor neuron diseases (MNDs) such as amyotrophic lateral sclerosis (ALS). As aging is a leading risk factor for the development of neurodegeneration, it is important to recapitulate age-related characteristics by using MNs at pathogenic ages. So far, cell reprogramming through induced pluripotent stem cells (iPSCs) and direct reprogramming from primary fibroblasts are two major strategies to obtain populations of MNs. While iPSC generation must go across the epigenetic landscape toward the pluripotent state, directly converted MNs might have the advantage of preserving aging-associated features from fibroblast donors...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29163029/micrornas-and-target-genes-as-biomarkers-for-the-diagnosis-of-early-onset-of-parkinson-disease
#5
REVIEW
Ahmad R Arshad, Siti A Sulaiman, Amalia A Saperi, Rahman Jamal, Norlinah Mohamed Ibrahim, Nor Azian Abdul Murad
Among the neurodegenerative disorders, Parkinson's disease (PD) ranks as the second most common disorder with a higher prevalence in individuals aged over 60 years old. Younger individuals may also be affected with PD which is known as early onset PD (EOPD). Despite similarities between the characteristics of EOPD and late onset PD (LODP), EOPD patients experience much longer disease manifestations and poorer quality of life. Although some individuals are more prone to have EOPD due to certain genetic alterations, the molecular mechanisms that differentiate between EOPD and LOPD remains unclear...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29163025/bdnf-trkb-induction-of-calcium-transients-through-cav2-2-calcium-channels-in-motoneurons-corresponds-to-f-actin-assembly-and-growth-cone-formation-on-%C3%AE-2-chain-laminin-221
#6
Benjamin Dombert, Stefanie Balk, Patrick Lüningschrör, Mehri Moradi, Rajeeve Sivadasan, Lena Saal-Bauernschubert, Sibylle Jablonka
Spontaneous Ca(2+) transients and actin dynamics in primary motoneurons correspond to cellular differentiation such as axon elongation and growth cone formation. Brain-derived neurotrophic factor (BDNF) and its receptor trkB support both motoneuron survival and synaptic differentiation. However, in motoneurons effects of BDNF/trkB signaling on spontaneous Ca(2+) influx and actin dynamics at axonal growth cones are not fully unraveled. In our study we addressed the question how neurotrophic factor signaling corresponds to cell autonomous excitability and growth cone formation...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29163013/motor-areas-show-altered-dendritic-structure-in-an-amyotrophic-lateral-sclerosis-mouse-model
#7
Matthew J Fogarty, Erica W H Mu, Nickolas A Lavidis, Peter G Noakes, Mark C Bellingham
Objective: Motor neurons (MNs) die in amyotrophic lateral sclerosis (ALS), a clinically heterogeneous neurodegenerative disease of unknown etiology. In human or rodent studies, MN loss is preceded by increased excitability. As increased neuronal excitability correlates with structural changes in dendritic arbors and spines, we have examined longitudinal changes in dendritic structure in vulnerable neuron populations in a mouse model of familial ALS. Methods: We used a modified Golgi-Cox staining method to determine the progressive changes in dendritic structure of hippocampal CA1 pyramidal neurons, striatal medium spiny neurons, and resistant (trochlear, IV) or susceptible (hypoglossal, XII; lumbar) MNs from brainstem and spinal cord of mice over-expressing the human SOD1(G93A) (SOD1) mutation, in comparison to wild-type (WT) mice, at four postnatal (P) ages of 8-15, 28-35, 65-75, and 120 days...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29162978/exploring-the-interaction-of-drosophila-tdp-43-and-the-type-ii-voltage-gated-calcium-channel-cacophony-in-regulating-motor-function-and-behavior
#8
Kayly M Lembke, David B Morton
Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neurodegenerative disease. The cause of the disease remains obscure, and as such there is no effective treatment or cure. Amyotrophic lateral sclerosis and other neurodegenerative diseases are frequently characterized by dysfunction of the RNA-binding protein, TDP-43. Using model systems to understand the mechanisms underlying TDP-43 dysfunction should accelerate identification of therapeutic targets. A recent report has shown that motor defects caused by the deletion of the Drosophila TDP-43 ortholog, tbph, are not driven by changes in the physiology at the neuromuscular junction...
2017: Journal of Experimental Neuroscience
https://www.readbyqxmd.com/read/29160009/mirna-in-spinal-muscular-atrophy-pathogenesis-and-therapy
#9
REVIEW
Francesca Magri, Fiammetta Vanoli, Stefania Corti
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by the selective death of lower motor neurons in the brain stem and spinal cord. SMA is caused by mutations in the survival motor neuron 1 gene (SMN1), leading to the reduced expression of the full-length SMN protein. microRNAs (miRNAs) are small RNAs that regulate post-transcriptional gene expression. Recent findings have suggested an important role for miRNAs in the pathogenesis of motor neuron diseases, including SMA...
November 21, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29159046/imaging-of-glia-activation-in-people-with-primary-lateral-sclerosis
#10
Sabrina Paganoni, Mohamad J Alshikho, Nicole R Zürcher, Paul Cernasov, Suma Babu, Marco L Loggia, James Chan, Daniel B Chonde, David Izquierdo Garcia, Ciprian Catana, Caterina Mainero, Bruce R Rosen, Merit E Cudkowicz, Jacob M Hooker, Nazem Atassi
Background: Glia activation is thought to contribute to neuronal damage in several neurodegenerative diseases based on preclinical and human post-mortem studies, but its role in primary lateral sclerosis (PLS) is unknown. Objectives: To localize and measure glia activation in people with PLS compared to healthy controls (HC). Methods: Ten participants with PLS and ten age-matched HCs underwent simultaneous magnetic resonance (MR) and proton emission tomography (PET)...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29158022/activation-of-the-nrf2-signaling-pathway-and-neuroprotection-of-nigral-dopaminergic-neurons-by-a-novel-synthetic-compound-kms99220
#11
Ji Ae Lee, Hyo Jin Son, Ji Won Choi, Jinwoo Kim, Se Hee Han, Nari Shin, Ji Hyun Kim, Soo Jeong Kim, Jun Young Heo, Dong Jin Kim, Ki Duk Park, Onyou Hwang
The transcription factor Nrf2 is known to induce gene expression of antioxidant enzymes and proteasome subunits. Because both oxidative stress and protein aggregation have damaging effects on neurons, activation of the Nrf2 signaling should be beneficial against neurodegeneration. In this study, we report a novel synthetic morpholine-containing chalcone KMS99220 that confers neuroprotection. It showed high binding affinity to the Nrf2 inhibitory protein Keap-1 and increased nuclear translocation of Nrf2 and gene expression of the antioxidant enzymes heme oxygenase-1, NAD(P)H:quinone oxidoreductase-1, and the catalytic and modifier subunits of glutamate-cysteine ligase in dopaminergic CATH...
November 17, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/29156920/supportive-palliative-interventions-in-motor-neurone-disease-what-we-know-from-current-literature
#12
Hon Wai Benjamin Cheng, Kwok Ying Chan, Yuen Kwan Judy Chung, Chun Wai Choi, Chun Hung Chan, Shuk Ching Cheng, Wan Hung Chan, Koon Sim Fung, Kar Yin Wong, Oi Man Iman Chan, Ching Wah Man
Although there is no cure for motor neurone disease (MND), the advent of supportive interventions including multidisciplinary care (MDC) has improved treatment interventions and enhanced quality of life (QOL) for MND patients and their carers. Our integrative review showed evidence-based MDC, respiratory management and disease-modifying therapy that have improved the outcomes of patients diagnosed with MND. Supportive approaches to nutritional maintenance and optimization of symptomatic treatments, including management of communication and neuropsychiatric issues, improve the QOL for MND patients...
October 31, 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29156917/advance-care-planning-for-patients-with-advanced-neurology-diseases
#13
Ka-Chi Cheung, Vikki Wai-Kee Lau, Ka-Chun Un, Man-Sheung Wong, Kwok-Ying Chan
BACKGROUND: Advanced neurology diseases including motor neuron disease (MND) are usually progressive life-limiting illness and could be devastating for patients, families and caregivers. Although medical technologies, such as enteral feeding and non-invasive ventilation, may prolong life expectancy of the patients, their utilization prompts important ethical questions in regard to their quality of life (QoL). Little attention had been paid on how ACP practice would practically help with patients suffering from different neurology diseases...
October 13, 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29156906/the-development-of-neurology-palliative-care-service-for-motor-neuron-disease-mnd-patients-hong-kong-experience
#14
Hon Wai Benjamin Cheng, Wai Tsan Tracy Chen, Chun Kwok Angus Chu, Savio Lee, Joo Shium Lee, Yeuk Fai Hong, Yuen Kwan Judy Chung
BACKGROUND: Motor neuron disease (MND) is a neurodegenerative disease characterized by loss of motor neurons in the spinal cord, brainstem and motor cortex. Clinically it is manifested as progressive decline in physical, respiratory, swallowing and communication function and ultimately death. Traditional model of care was fragmented and did not match with patients and carers multi-facet needs. METHODS: A special workgroup for MND patients that includes neurologist, respiratory physician, rehabilitation specialist and palliative care (PC) physician was formed in Hong Kong since year 2013...
September 15, 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29156412/a-system-level-mathematical-model-of-basal-ganglia-motor-circuit-for-kinematic-planning-of-arm-movements
#15
Armin Salimi-Badr, Mohammad Mehdi Ebadzadeh, Christian Darlot
In this paper, a novel system-level mathematical model of the Basal Ganglia (BG) for kinematic planning, is proposed. An arm composed of several segments presents a geometric redundancy. Thus, selecting one trajectory among an infinite number of possible ones requires overcoming redundancy, according to some kinds of optimization. Solving this optimization is assumed to be the function of BG in planning. In the proposed model, first, a mathematical solution of kinematic planning is proposed for movements of a redundant arm in a plane, based on minimizing energy consumption...
November 10, 2017: Computers in Biology and Medicine
https://www.readbyqxmd.com/read/29154925/membrane-cholesterol-depletion-in-cortical-neurons-highlights-altered-nmda-receptor-functionality-in-a-mouse-model-of-amyotrophic-lateral-sclerosis
#16
Alessia Antonini, Silvia Caioli, Luana Saba, Giulia Vindigni, Silvia Biocca, Nadia Canu, Cristina Zona
Amyotrophic Lateral Sclerosis (ALS) is a chronic neurodegenerative disease affecting upper and lower motor neurons, with unknown aetiology. Lipid rafts, cholesterol enriched microdomains of the plasma membrane, have been linked to neurodegenerative disorders like ALS. The NMDA-receptor subcellular localization in lipid rafts is known to play many roles, from modulating memory strength to neurotoxicity. In this study, performed on the widely used G93A mouse model of ALS, we have shown an equal content of total membrane cholesterol in Control and G93A cortical cultures...
November 14, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29154923/dissociation-of-blood-brain-barrier-disruption-and-disease-manifestation-in-an-aquaporin-4-deficient-mouse-model-of-amyotrophic-lateral-sclerosis
#17
Saori Watanabe-Matsumoto, Yasuhiro Moriwaki, Takashi Okuda, Shinji Ohara, Koji Yamanaka, Yoichiro Abe, Masato Yasui, Hidemi Misawa
Aquaporin-4 (AQP4) is abundantly expressed in the central nervous system and is involved in the water balance in the cellular environment. Previous studies have reported that AQP4 expression is upregulated in rat models of amyotrophic lateral sclerosis (ALS), a fatal disease that affects motor neurons in the brain and spinal cord. In this study, we report that astrocytic AQP4 overexpression is evident during the course of disease in the spinal cord of an ALS mouse model, as well as in tissue from patients with ALS...
November 15, 2017: Neuroscience Research
https://www.readbyqxmd.com/read/29154281/gradual-cerebral-hypoperfusion-impairs-fear-conditioning-and-object-recognition-learning-and-memory-in-mice-potential-roles-of-neurodegeneration-and-cholinergic-dysfunction
#18
Jogender Mehla, Sean Lacoursiere, Emily Stuart, Robert J McDonald, Majid H Mohajerani
In the present study, male C57BL/6J mice were subjected to gradual cerebral hypoperfusion by implanting an ameroid constrictor (AC) on the left common carotid artery (CCA) and a stenosis on the right CCA. In the sham group, all surgical procedures were kept the same except no AC was implanted and stenosis was not performed. One month following the surgical procedures, fear conditioning and object recognition tests were conducted to evaluate learning and memory functions and motor functions were assessed using a balance beam test...
November 14, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29154076/multiple-intracerebroventricular-injections-of-human-umbilical-cord-mesenchymal-stem-cells-delay-motor-neurons-loss-but-not-disease-progression-of-sod1g93a-mice
#19
Francesca Sironi, Antonio Vallarola, Martina Bruna Violatto, Laura Talamini, Mattia Freschi, Roberta De Gioia, Chiara Capelli, Azzurra Agostini, Davide Moscatelli, Massimo Tortarolo, Paolo Bigini, Martino Introna, Caterina Bendotti
Stem cell therapy is considered a promising approach in the treatment of amyotrophic lateral sclerosis (ALS) and mesenchymal stem cells (MSCs) seem to be the most effective in ALS animal models. The umbilical cord (UC) is a source of highly proliferating fetal MSCs, more easily collectable than other MSCs. Recently we demonstrated that human (h) UC-MSCs, double labeled with fluorescent nanoparticles and Hoechst-33258 and transplanted intracerebroventricularly (ICV) into SOD1G93A transgenic mice, partially migrated into the spinal cord after a single injection...
November 10, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29154038/dna-damage-and-neurodegenerative-phenotypes-in-aged-ciz1-null-mice
#20
Mohammad Moshahid Khan, Jianfeng Xiao, Damini Patel, Mark S LeDoux
Cell-cycle dysfunction and faulty DNA repair are closely intertwined pathobiological processes that may contribute to several neurodegenerative disorders. CDKN1A interacting zinc finger protein 1 (CIZ1) plays a critical role in DNA replication and cell-cycle progression at the G1/S checkpoint. Germline or somatic variants in CIZ1 have been linked to several neural and extra-neural diseases. Recently, we showed that germline knockout of Ciz1 is associated with motor and hematological abnormalities in young adult mice...
November 16, 2017: Neurobiology of Aging
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