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https://www.readbyqxmd.com/read/28895473/neuroinflammation-in-amyotrophic-lateral-sclerosis-role-of-redox-dys-regulation
#1
Nadia D'Ambrosi, Mauro Cozzolino, Maria Teresa Carrì
SIGNIFICANCE: Amyotrophic lateral sclerosis (ALS) is due to degeneration of upper and lower motor neurons in the anterior horn of the spinal cord and in the motor cortex. Mechanisms leading to motor neuron death are complex and currently the disease is untreatable. Recent Advances. Work in genetic models of ALS indicates that an imbalance in the crosstalk that physiologically exists between motor neurons and the surrounding cells is eventually detrimental to motor neurons. In particular, the cascade of events collectively known as neuroinflammation and mainly characterized by a reactive phenotype of astrocytes and microglia, moderate infiltration of peripheral immune cells and elevated levels of inflammatory mediators has been consistently observed in motor regions of the central nervous system in sporadic and familial ALS, constituting a hallmark of the disease...
September 12, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28889642/treating-pediatric-neuromuscular-disorders-the-future-is-now
#2
REVIEW
James J Dowling, Hernan D Gonorazky, Ronald D Cohn, Craig Campbell
Pediatric neuromuscular diseases encompass all disorders with onset in childhood and where the primary area of pathology is in the peripheral nervous system. These conditions are largely genetic in etiology, and only those with a genetic underpinning will be presented in this review. This includes disorders of the anterior horn cell (e.g., spinal muscular atrophy), peripheral nerve (e.g., Charcot-Marie-Tooth disease), the neuromuscular junction (e.g., congenital myasthenic syndrome), and the muscle (myopathies and muscular dystrophies)...
September 10, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28884921/survival-beyond-the-perinatal-period-expands-the-phenotypes-caused-by-mutations-in-gle1
#3
Edith Said, Jessica X Chong, Maja Hempel, Jonas Denecke, Paul Soler, Tim Strom, Deborah A Nickerson, Christian Kubisch, Michael J Bamshad, Davor Lessel
Mutations in GLE1 underlie Lethal Congenital Contracture syndrome (LCCS) and Lethal Arthrogryposis with Anterior Horn Cell Disease (LAAHD). Both LCCS and LAAHD are characterized by reduced fetal movements, congenital contractures, and a severe form of motor neuron disease that results in fetal death or death in the perinatal period, respectively. We identified bi-allelic mutations in GLE1 in two unrelated individuals with motor delays, feeding difficulties, and respiratory insufficiency who survived beyond the perinatal period...
September 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28867325/neuroprotective-effects-of-valproic-acid-in-a-rat-model-of-cauda-equina-injury
#4
Qing-Jie Kong, Yuan Wang, Yang Liu, Jing-Chuan Sun, Xi-Ming Xu, Xiao-Fei Sun, Jian-Gang Shi
BACKGROUND: Histone deacetylase inhibitors (HDACi), including valproic acid (VPA), are promising therapeutic interventions in neurological disorders and play an important role in synaptic activity and neuronal function. MATERIAL AND METHODS: A total of 30 rats were randomly allocated to three groups: sham, control, and VPA. The rats in the VPA and control groups received laminectomy at the L4 level of the vertebrae and silicone gel implantation into the epdidural spaces L5 and L6...
August 31, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28853070/activation-of-microglyocytes-in-the-anterior-horns-of-rat-spinal-cord-after-administration-of-bacterial-lipopolysaccharide
#5
E A Kolos, D E Korzhevskii
We studied the reaction of the microglia of the anterior horns of the rat spinal cord to intraperitoneal administration of bacterial LPS. Immunohistochemical analysis showed that acute systemic inflammation leads to activation of more than half of microglial cells as soon as in 24 h after LPS injection, while the total number of microglial cells does not change significantly. It was hypothesized that activated microglial cells are involved in the reorganization of synaptic connections, but do not have a neurotoxic effect...
August 29, 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28829151/validation-of-an-immunohistochemistry-assay-for-detection-of-cd155-the-poliovirus-receptor-in-malignant-gliomas
#6
Vidyalakshmi Chandramohan, Jeffrey D Bryant, Hailan Piao, Stephen T Keir, Eric S Lipp, Michaela Lefaivre, Kathryn Perkinson, Darell D Bigner, Matthias Gromeier, Roger E McLendon
CONTEXT: - The oncolytic polio-rhinovirus recombinant (PVSRIPO) has demonstrated promise in currently ongoing phase I/II clinical trials against recurrent glioblastoma and was granted breakthrough therapy designation by the Food and Drug Administration/Center for Biologics Evaluation and Research. A reliable clinical assay to document expression of the poliovirus receptor, CD155, in routinely available patient tumor samples is needed for continued clinical development of PVSRIPO oncolytic immunotherapy in primary brain tumors and beyond...
August 22, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28798922/practical-approach-to-the-patient-with-acute-neuromuscular-weakness
#7
REVIEW
Rajeev Nayak
Acute neuromuscular paralysis (ANMP) is a clinical syndrome characterized by rapid onset muscle weakness progressing to maximum severity within several days to weeks (less than 4 wk). Bulbar and respiratory muscle weakness may or may not be present. It is a common neurological emergency which requires immediate and careful investigations to determine the etiology because accurate diagnosis has significant impact on therapy and prognosis. Respiratory failure caused by neuromuscular weakness is considered as more critical than lung disease because its development may be insidious or subtle until sudden decompensation leads to life threatening hypoxia...
July 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28766957/immunohistochemical-detection-of-c9orf72-protein-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-patterns-of-immunostaining-and-an-evaluation-of-commercial-antibodies
#8
Yvonne S Davidson, Andrew C Robinson, Sara Rollinson, Stuart Pickering-Brown, Shangxi Xiao, Janice Robertson, David M A Mann
We have employed as 'gold standards' two in-house, well-characterised and validated polyclonal antibodies, C9-L and C9-S, which detect the longer and shorter forms of C9orf72, and have compared seven other commercially available antibodies with these in order to evaluate the utility of the latter as credible tools for the demonstration of C9orf72. C9-L and C9-S antibodies immunostained cytoplasmic 'speckles', and the nuclear membrane, respectively, in cerebellar Purkinje cells of the cerebellum in patients with behavioural variant frontotemporal dementia (bvFTD) with amyotrophic lateral sclerosis (ALS), and in patients with ALS alone...
August 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28749553/myelopathy-in-beh%C3%A3-et-s-disease-the-bagel-sign
#9
Ugur Uygunoglu, Burcu Zeydan, Yesim Ozguler, Serdal Ugurlu, Emire Seyahi, Naci Kocer, Civan Islak, Kejal Kantarci, Sabahattin Saip, Aksel Siva, Orhun H Kantarci
OBJECTIVE: To describe the clinical and distinctive imaging features of myelopathy associated with Behçet's disease (BD). METHODS: We evaluated the records of patients meeting the following criteria: (1) fulfillment of the International Study Group criteria for BD; (2) clinically suggestive of myelopathy; (3) simultaneous spinal cord and brain magnetic resonance images (MRIs) within 1 month of acute worsening of myelopathy; and (4) follow-up duration ≥ 1 year after initial MRI evaluation...
August 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28729373/the-importance-of-managing-the-patient-and-not-the-gene-expanded-phenotype-of-gle1-associated-arthrogryposis
#10
Queenie K-G Tan, Allyn McConkie-Rosell, Jane Juusola, Kathryn E Gustafson, Carolyn E Pizoli, Anne F Buckley, Yong-Hui Jiang
GLE1 encodes a protein important for mRNA export and appears to play roles in translation initiation and termination as well. Pathogenic variants in GLE1 mutations have been associated with lethal contracture syndrome (LCCS1) and Lethal Arthrogryposis with Anterior Horn Cell Disease (LAAHD); phenotypes reported in individuals include fetal akinesia and a severe form of motor neuron disease, typically presenting with prenatal symptoms and perinatal lethality. In this paper, we identified bi-allelic missense mutations in GLE1 by trio whole exome sequencing (WES) in an individual affected with congenital motor weakness and contractures as well as feeding and respiratory difficulties...
July 20, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28669296/a-neurophysiological-approach-to-nerve-transfer-to-restore-upper-limb-function-in-cervical-spinal-cord-injury
#11
Ross M Mandeville, Justin M Brown, Geoffrey L Sheean
A successful nerve transfer surgery can provide a wealth of benefits to a patient with cervical spinal cord injury. The process of surgical decision making ideally uses all pertinent information to produce the best functional outcome. Reliance on clinical examination and imaging studies alone can miss valuable information on the state of spinal cord health. In this regard, neurophysiological evaluation has the potential to effectively gauge the neurological status of even select pools of anterior horn cells and their axons to small nerve branches in question to determine the potential efficacy of their use in a transfer...
July 2017: Neurosurgical Focus
https://www.readbyqxmd.com/read/28657126/a-homozygous-i684t-in-gle1-as-a-novel-cause-of-arthrogryposis-and-motor-neuron-loss
#12
Teija Paakkola, Katri Vuopala, Hannaleena Kokkonen, Jaakko Ignatius, Marita Valkama, Jukka S Moilanen, Somayyeh Fahiminiya, Jacek Majewski, Reetta Hinttala, Johanna Uusimaa
Mutations in GLE1, RNA export mediator (GLE1) gene have previously been shown to cause motor neuron diseases such as Lethal congenital contracture syndrome 1 (LCCS1) and Lethal arthrogryposis with anterior horn cell disease (LAAHD), including arthrogryposis, fetal akinesis and motor neuron loss as common clinical features. The homozygous FinMajor mutation p.T144_E145insPFQ has been described as one of the causes for LCCS1 whereas LAAHD is caused by a heterocompound FinMajor mutation together with p.R569H, p...
June 28, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28632899/novel-intracytoplasmic-inclusions-immunoreactive-for-phosphorylated-tdp43-and-cystatin-c-in-anterior-horn-cells-in-a-case-of-sporadic-amyotrophic-lateral-sclerosis
#13
Masayuki Shintaku, Daita Kaneda, Kiyomitsu Oyanagi
Novel intracytoplasmic inclusions immunoreactive for phosphorylated transactivation response DNA-binding protein 43 (p-TDP43), cystatin C, and transferrin were found in anterior horn cells in a case of sporadic amyotrophic lateral sclerosis (ALS). The patient was a 59-year-old woman, who died of ALS after a clinical course of 8 years. She had been receiving mechanical support for respiration for 6 years and in a "totally locked-in" state for 4 years prior to death. The spinal cord showed severe degeneration involving the anterior and lateral funiculi, whereas the posterior funiculus was preserved...
June 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28630311/a-prokaryotic-viral-sequence-is-expressed-and-conserved-in-mammalian-brain
#14
Yang-Hui Yeh, Vignesh Gunasekharan, Laura Manuelidis
A natural and permanent transfer of prokaryotic viral sequences to mammals has not been reported by others. Circular "SPHINX" DNAs <5 kb were previously isolated from nuclease-protected cytoplasmic particles in rodent neuronal cell lines and brain. Two of these DNAs were sequenced after Φ29 polymerase amplification, and they revealed significant but imperfect homology to segments of commensal Acinetobacter phage viruses. These findings were surprising because the brain is isolated from environmental microorganisms...
July 3, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28584672/keratoacanthoma-of-the-nasal-septum-secondary-to-ranibizumab-use
#15
Jason E Cohn, Hilary M Caruso Sales, Giang Huong Nguyen, Harvey Spector, Kenneth Briskin
Keratoacanthoma (KA) is a benign epithelial tumor that typically presents as a firm, cone-shaped, flesh-colored nodule with a central horn-filled crater. KA is considered to be a low-grade variant of squamous cell carcinoma (SCC). We report a rare case of a 72-year-old male who presented with a KA involving the nasal septum, possibly related to ranibizumab use. A flesh-colored lesion on the right anterior nasal septum lesion was visualized on examination. Histologic examination revealed a well-circumscribed, dome-shaped central crater filled with keratin, well-differentiated squamous epithelium with ground-glass cytoplasm with pushing margins, and intraepithelial microabscesses establishing the diagnosis of KA...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28554554/expanding-the-phenotypic-spectrum-associated-with-mutations-of-dync1h1
#16
Sarah J Beecroft, Catriona A McLean, Martin B Delatycki, Kurian Koshy, Eppie Yiu, Goknur Haliloglu, Diclehan Orhan, Phillipa J Lamont, Mark R Davis, Nigel G Laing, Gianina Ravenscroft
Autosomal dominant mutations of DYNC1H1 cause a range of neurogenetic diseases, including mental retardation with cortical malformations, hereditary spastic paraplegia and spinal muscular atrophy. Using SNP array, linkage analysis and next generation sequencing, we identified two families and one isolated proband sharing a known spinal muscular atrophy, lower extremity predominant (SMALED) causing mutation DYNC1H1 c.1792C>T, p.Arg598Cys, and another family harbouring a c.2327C>T, p.Pro776Leu mutation...
July 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28549966/lentivirus-mediated-inhibition-of-aqp4-accelerates-motor-function-recovery-associated-with-ngf-in-spinal-cord-contusion-rats
#17
Jianmin Chen, Xi Zeng, Shengnan Li, Zhanqiong Zhong, Xi Hu, Hongyu Xiang, Ying Rao, Lixin Zhang, Xiuya Zhou, Qingjie Xia, Tinghua Wang, Xiao Zhang
Aquaporin-4 (AQP4) is a water channel protein in spinal cord and plays a critical role in the pathophysiological process of spinal cord injury (SCI). However, little is known about the molecular mechanism of AQP4 involved in SCI. The present study was performed to investigate the possible molecules regulated by AQP4 after SCI by use of lentiviral-mediated RNA interference (RNAi). First, the motor function was evaluated by Basso, Beattie, Bresnahan (BBB) scale and the expression of AQP4 was measured by western blot, immunohistochemical staining and immunofluorescence in rats after contusion spinal cord injury (cSCI)...
May 23, 2017: Brain Research
https://www.readbyqxmd.com/read/28539831/aldehyde-dehydrogenases-1a2-expression-and-distribution-are-potentially-associated-with-neuron-death-in-spinal-cord-of-tg-sod1-g93a-1gur-mice
#18
Huiting Liang, Chengsi Wu, Youqing Deng, Lei Zhu, Jie Zhang, Weiming Gan, Chunyan Tang, Renshi Xu
The pathogenesis of amyotrophic lateral sclerosis (ALS) has not been unclear yet, it might be associated with the abnormal expression and distribution of certain proteins. Aldehyde dehydrogenases 1A2 (ALDH1A2) was thought to be one of potential candidates. Therefore, in this study we observed and analyzed the alteration of the expression and distribution of ALDH1A2 in the spinal cord of wild-type (WT) and Tg(SOD1*G93A)1Gur mice. We compared the expression and distribution of ALDH1A2 in the different segments, anatomic regions and neural cells of spinal cord at the different stages of WT and Tg(SOD1*G93A)1Gur mice applied the methods of fluorescent immunohistochemistry and western blot...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28528422/5-ala-fluorescence-guided-endoscopic-surgery-for-mixed-germ-cell-tumors
#19
Junichi Takeda, Masahiro Nonaka, Yi Li, Yumiko Komori, Takamasa Kamei, Ryoichi Iwata, Tetsuo Hashiba, Kunikazu Yoshimura, Akio Asai
5-Aminolevulinic acid (5-ALA) fluorescence-guided surgery is widely used for detection and planning of resection of malignant gliomas and other brain tumors. However, no reports have described 5-ALA fluorescence-guided surgery or direct visualization of germ cell tumors. Here, we report two cases of germ cell tumors in which a positive 5-ALA fluorescent signal was visualized with a neuroendoscope. Both cases had a tumor in the pineal region that was associated with hydrocephalus. The patients underwent surgery after administration of 5-ALA...
August 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28413866/inositol-hexakisphosphate-kinase-2-is-a-presymptomatic-biomarker-for-amyotrophic-lateral-sclerosis
#20
Yusuke Moriya, Eiichiro Nagata, Natsuko Fujii, Tadayuki Satoh, Haruko Ogawa, Shinji Hadano, Shunya Takizawa
OBJECTIVE: Inositol hexakisphosphate kinase 2 (InsP6K2), an enzyme that converts inositol hexakisphosphate (InsP6) to diphosphoinositol pentakisphosphate (InsP7), induces cell death. InsP6K2 is abundant in the central nervous system, especially anterior horn cells of spinal cord. To identify the role of InsP6K2 in amyotrophic lateral sclerosis (ALS), we investigated the expression levels of InsP6K2 in transgenic mice expressing mutant superoxide dismutase-1 (SOD1) (mSOD1 Tg mice). METHODS: The specimens of spinal cords were obtained from mSOD1 Tg mice and age-matched wild-type mice...
April 20, 2017: Tokai Journal of Experimental and Clinical Medicine
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