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https://www.readbyqxmd.com/read/28108290/pmp22-mutant-allele-specific-sirna-alleviates-demyelinating-neuropathic-phenotype-in-vivo
#1
Ji-Su Lee, Eun Hyuk Chang, Ok Jae Koo, Dong Hwan Jwa, Won Min Mo, Geon Kwak, Hyo Won Moon, Hwan Tae Park, Young Bin Hong, Byung-Ok Choi
Charcot-Marie-Tooth disease (CMT) is a genetic disorder that can be caused by aberrations in >80 genes. CMT has heterogeneous modes of inheritance, including autosomal dominant, autosomal recessive, X-linked dominant, and X-linked recessive. Over 95% of cases are dominantly inherited. In this study, we investigated whether regulation of a mutant allele by an allele-specific small interfering RNA (siRNA) can alleviate the demyelinating neuropathic phenotype of CMT. We designed 19 different allele-specific siRNAs for Trembler J (Tr-J) mice harboring a naturally occurring mutation (Leu16Pro) in Pmp22...
January 17, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28108255/regular-exercise-promotes-memory-function-and-enhances-hippocampal-neuroplasticity-in-experimental-autoimmune-encephalomyelitis-mice
#2
Tae-Woon Kim, Yun-Hee Sung
Multiple sclerosis (MS) is a progressive condition affecting the central nervous system (CNS), and is characterized by the development of demyelinated lesions and plaques in the brain and spinal cord. Exercise is beneficial against dementia in elderly patients, so we investigated the effects of exercise on memory in relation to hippocampal demyelination and neuroplasticity in a mouse model of MS (experimental autoimmune encephalomyelitis [EAE]). Mice were randomly divided into three groups: Sham, EAE, and EAE and exercise (EAE+EX)...
January 17, 2017: Neuroscience
https://www.readbyqxmd.com/read/28107629/discovery-of-1-2-3-triazole-derivatives-for-multimodality-pet-ct-cryoimaging-of-myelination-in-the-central-nervous-system
#3
Chunying Wu, Brendan Eck, Sheng Zhang, Junqing Zhu, Anand Dev Tiwari, Yifan Zhang, Yunjie Zhu, Jinming Zhang, Bin Wang, Xizhen Wang, Xu Wang, Jingqiang You, Jian Wang, Yihui Guan, Xingdang Liu, Xin Yu, Bruce D Trapp, Robert Miller, Jerry Silver, David Wilson, Yanming Wang
Myelin pathology is present in many neurological conditions such as multiple sclerosis (MS) and traumatic spinal cord injury (SCI). To facilitate development of novel therapies aimed at myelin repair, we set out to develop imaging agents that permit direct quantification of myelination in vivo. In this work, we designed and synthesized a series of fluorescent fluorinated myelin imaging agents that can be used for in vivo positron emission tomography (PET) imaging combined with subsequent post-mortem fluorescent cryoimaging...
January 20, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28107514/karyopherin-alpha-proteins-regulate-oligodendrocyte-differentiation
#4
Benjamin M Laitman, John N Mariani, Chi Zhang, Setsu Sawai, Gareth R John
Proper regulation of the coordinated transcriptional program that drives oligodendrocyte (OL) differentiation is essential for central nervous system myelin formation and repair. Nuclear import, mediated in part by a group of karyopherin alpha (Kpna) proteins, regulates transcription factor access to the genome. Understanding how canonical nuclear import functions to control genomic access in OL differentiation may aid in the creation of novel therapeutics to stimulate myelination and remyelination. Here, we show that members of the Kpna family regulate OL differentiation, and may play distinct roles downstream of different pro-myelinating stimuli...
2017: PloS One
https://www.readbyqxmd.com/read/28106136/long-term-exposure-to-835%C3%A2-mhz-rf-emf-induces-hyperactivity-autophagy-and-demyelination-in-the-cortical-neurons-of-mice
#5
Ju Hwan Kim, Da-Hyeon Yu, Yang Hoon Huh, Eun Ho Lee, Hyung-Gun Kim, Hak Rim Kim
Radiofrequency electromagnetic field (RF-EMF) is used globally in conjunction with mobile communications. There are public concerns of the perceived deleterious biological consequences of RF-EMF exposure. This study assessed neuronal effects of RF-EMF on the cerebral cortex of the mouse brain as a proxy for cranial exposure during mobile phone use. C57BL/6 mice were exposed to 835 MHz RF-EMF at a specific absorption rate (SAR) of 4.0 W/kg for 5 hours/day during 12 weeks. The aim was to examine activation of autophagy pathway in the cerebral cortex, a brain region that is located relatively externally...
January 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28106069/identification-of-novel-cellular-clusters-define-a-specialized-area-in-the-cerebellar-periventricular-zone
#6
María Alejandra González-González, Gabriela B Gómez-González, Marymar Becerra-González, Ataúlfo Martínez-Torres
The periventricular zone of cerebellum is a germinative niche during the embryonic development, nevertheless its structural organization and functional implications in adult have not been widely studied. Here we disclose the presence of two novel clusters of cells in that area. The first one was named the subventricular cellular cluster (SVCC) and is composed of cells that express glial and neuronal markers. The second was named the ventromedial cord (VMC) and appears as a streak of biciliated cells with microvillosities facing the ventricle, that includes GFAP(+) and nestin(+) cells organized along the periventricular vasculature...
January 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28105588/nogo-a-antibodies-for-progressive-multiple-sclerosis
#7
Benjamin V Ineichen, Patricia S Plattner, Nicolas Good, Roland Martin, Michael Linnebank, Martin E Schwab
Most of the current therapies, as well as many of the clinical trials, for multiple sclerosis (MS) target the inflammatory autoimmune processes, but less than 20% of all clinical trials investigate potential therapies for the chronic progressive disease stage of MS. The latter is responsible for the steadily increasing disability in many patients, and there is an urgent need for novel therapies that protect nervous system tissue and enhance axonal growth and/or remyelination. As outlined in this review, solid pre-clinical data suggest neutralization of the neurite outgrowth inhibitor Nogo-A as a potential new way to achieve both axonal and myelin repair...
January 19, 2017: CNS Drugs
https://www.readbyqxmd.com/read/28105459/mog-antibody-positive-benign-unilateral-cerebral-cortical-encephalitis-with-epilepsy
#8
Ryo Ogawa, Ichiro Nakashima, Toshiyuki Takahashi, Kimihiko Kaneko, Tetsuya Akaishi, Yoshiki Takai, Douglas Kazutoshi Sato, Shuhei Nishiyama, Tatsuro Misu, Hiroshi Kuroda, Masashi Aoki, Kazuo Fujihara
OBJECTIVE: To describe the features of adult patients with benign, unilateral cerebral cortical encephalitis positive for the myelin oligodendrocyte glycoprotein (MOG) antibody. METHODS: In this retrospective, cross-sectional study, after we encountered an index case of MOG antibody-positive unilateral cortical encephalitis with epileptic seizure, we tested for MOG antibody using our in-house, cell-based assay in a cohort of 24 consecutive adult patients with steroid-responsive encephalitis of unknown etiology seen at Tohoku University Hospital (2008-2014)...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28104252/novel-zebrafish-eae-model-a-quick-in-vivo-screen-for-multiple-sclerosis
#9
Pushkar Kulkarni, Swapna Yellanki, Raghavender Medishetti, Dharmarajan Sriram, Uday Saxena, Perumal Yogeeswari
INTRODUCTION: Pre-clinical drug discovery for multiple sclerosis (MS) is a labor intensive activity to perform in rodent models. This is owing to the long duration of disease induction and observation of treatment effects in an experimental autoimmune encephalomyelitis (EAE) model. We propose a novel adult zebrafish based model which offers a quick and simple protocol that can used to screen candidates as a step between in vitro experiments and rodent studies. The experiments conducted for this manuscript were to standardize a suitable model of EAE in adult zebrafish and validate it using known modulators...
January 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28103473/lnc-edin-to-myelin
#10
Stacey M Glasgow, Benjamin Deneen
Long non-coding RNAs (lncRNAs) have been implicated in numerous developmental processes. In a technical and bioinformatics tour-de-force, He et al. (2017) provide critical insight into the dynamics of lncRNA expression, function, and mechanism during oligodendrocyte development and after injury.
January 18, 2017: Neuron
https://www.readbyqxmd.com/read/28103057/distribution-pattern-of-dorsal-root-ganglion-neurons-synthesizing-nitric-oxide-synthase-in-different-animal-species
#11
Dalibor Kolesár, Mária Kolesárová, Ján Kyselovič
The main aim of the present review is to provide at first a short survey of the basic anatomical description of sensory ganglion neurons in relation to cell size, conduction velocity, thickness of myelin sheath, and functional classification of their processes. In addition, we have focused on discussing current knowledge about the distribution pattern of neuronal nitric oxide synthase containing sensory neurons especially in the dorsal root ganglia in different animal species; hence, there is a large controversy in relation to interpretation of the results dealing with this interesting field of research...
November 24, 2016: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/28102945/learning-based-deformable-image-registration-for-infant-mr-images-in-the-first-year-of-life
#12
Shunbo Hu, Lifang Wei, Yaozong Gao, Yanrong Guo, Guorong Wu, Dinggang Shen
PURPOSE: Many brain development studies have been devoted to investigate dynamic structural and functional changes in the first year of life. To quantitatively measure brain development in such a dynamic period, accurate image registration for different infant subjects with possible large age gap is of high demand. Although many state-of-the-art image registration methods have been proposed for young and elderly brain images, very few registration methods work for infant brain images acquired in the first year of life, because of (a) large anatomical changes due to fast brain development and (b) dynamic appearance changes due to white-matter myelination...
January 2017: Medical Physics
https://www.readbyqxmd.com/read/28102813/intramedullary-amputation-neuroma-a-case-report-and-review-of-the-literature
#13
Laura Stone McGuire, Mandana Behbahini, Sumit Das, David Loeffler, Peter Burger, Herbert Engelhard, Tibor Valyi-Nagy, Ankit Mehta
BACKGROUND AND IMPORTANCE: Amputation neuromas consist of non-neoplastic collections of myelinated axons and Schwann cells and typically arise in injured peripheral nerves. Rarely, however, neuromas occur within the spinal cord. Intramedullary amputation neuromas have been described both with and without a history of trauma within the peripheral nervous system. We report a rare case of an isolated intramedullary spinal cord amputation neuroma. CLINICAL PRESENTATION: This 43-year-old man presented with progressive and severe gait deterioration for ~ 7 years...
January 19, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28102529/recovery-from-chemotherapy-induced-white-matter-changes-in-young-breast-cancer-survivors
#14
Thibo Billiet, Louise Emsell, Mathieu Vandenbulcke, Ronald Peeters, Daan Christiaens, Alexander Leemans, Wim Van Hecke, Ann Smeets, Frederic Amant, Stefan Sunaert, Sabine Deprez
In a previous longitudinal diffusion tensor imaging (DTI) study, we observed cerebral white matter (WM) alterations (reduced fractional anisotropy (FA)) related to decreased cognitive performance 3-5 months after chemotherapy-treatment (t2) when compared to baseline (t1) (Deprez et al. in Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology, 30(3), 274-281. doi:10.1200/JCO.2011.36.8571, 2012). The current study investigates the evolution and the nature of these previously observed microstructural changes...
January 19, 2017: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/28102462/cerebellar-pathways-in-mouse-model-of-purkinje-cell-degeneration-detected-by-high-angular-resolution-diffusion-imaging-tractography
#15
Yuri Kanamaru, Jianxue Li, Natalie Stewart, Richard L Sidman, Emi Takahashi
Cerebellar MR imaging has several challenging aspects, due to the fine, repetitive layered structure of cortical folia with underlying axonal pathways. In this MR study, we imaged with high-angular resolution diffusion imaging (HARDI) abnormal cerebellar cortical structure (gray matter) and myelinated axonal pathways (white matter) of a mouse spontaneous mutation, Purkinje cell degeneration (pcd), in which almost all Purkinje neurons degenerate, mainly between postnatal days 20 and 35. Mouse brains at postnatal day 20 (P20) and at 8 months were scanned, and known or expected abnormalities, such as reduction of the white matter volume, disorganized pathways likely linked to parallel fibers, mossy fibers, and other fibers running from/to the cerebellar cortex were observed in mutant mice...
January 19, 2017: Cerebellum
https://www.readbyqxmd.com/read/28101995/cholinergic-signaling-in-myelination
#16
REVIEW
R Douglas Fields, Dipankar J Dutta, Jillian Belgrad, Maya Robnett
There is a long history of research on acetylcholine (ACh) function in myelinating glia, but a resurgence of interest recently as a result of the therapeutic potential of manipulating ACh signaling to promote remyelination, and the broader interest in neurotransmitter signaling in activity-dependent myelination. Myelinating glia express all the major types of muscarinic and nicotinic ACh receptors at different stages of development, and acetylcholinesterase and butyrylcholinesterase are highly expressed in white matter...
January 19, 2017: Glia
https://www.readbyqxmd.com/read/28100454/gene-therapy-targeting-oligodendrocytes-provides-therapeutic-benefit-in-a-leukodystrophy-model
#17
Elena Georgiou, Kyriaki Sidiropoulou, Jan Richter, Christos Papaneophytou, Irene Sargiannidou, Alexia Kagiava, Georg von Jonquieres, Christina Christodoulou, Matthias Klugmann, Kleopas A Kleopa
Pelizaeus-Merzbacher-like disease or hypomyelinating leukodystrophy-2 is an autosomal recessively inherited leukodystrophy with childhood onset resulting from mutations in the gene encoding the gap junction protein connexin 47 (Cx47, encoded by GJC2). Cx47 is expressed specifically in oligodendrocytes and is crucial for gap junctional communication throughout the central nervous system. Previous studies confirmed that a cell autonomous loss-of-function mechanism underlies hypomyelinating leukodystrophy-2 and that transgenic oligodendrocyte-specific expression of another connexin, Cx32 (GJB1), can restore gap junctions in oligodendrocytes to achieve correction of the pathology in a disease model...
January 18, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28100104/theoretical-predication-of-temperature-effects-on-accommodative-processes-in-simulated-amyotrophic-lateral-sclerosis-during-hypothermia-and-hyperthermia
#18
D I Stephanova, A Kossev
Electrotonic potentials allow the accommodative processes to long-lasting subthreshold polarizing stimuli to be assessed. The present study investigates such potentials in previously simulated cases of amyotrophic lateral sclerosis, termed as ALS1, ALS2 and ALS3, respectively, when the temperature is changed during hypothermia ([Formula: see text]C) and hyperthermia ([Formula: see text]C). The ALS cases are modeled as three progressively severe uniform axonal dysfunctions along the human motor nerve fiber which is simulated by our temperature-dependent multi-layered numerical model...
January 18, 2017: Journal of Integrative Neuroscience
https://www.readbyqxmd.com/read/28096400/defining-recovery-neurobiology-of-injured-spinal-cord-by-synthetic-matrix-assisted-hmsc-implantation
#19
Alexander E Ropper, Devang K Thakor, InBo Han, Dou Yu, Xiang Zeng, Jamie E Anderson, Zaid Aljuboori, Soo-Woo Kim, Hongjun Wang, Richard L Sidman, Ross D Zafonte, Yang D Teng
Mesenchymal stromal stem cells (MSCs) isolated from adult tissues offer tangible potential for regenerative medicine, given their feasibility for autologous transplantation. MSC research shows encouraging results in experimental stroke, amyotrophic lateral sclerosis, and neurotrauma models. However, further translational progress has been hampered by poor MSC graft survival, jeopardizing cellular and molecular bases for neural repair in vivo. We have devised an adult human bone marrow MSC (hMSC) delivery formula by investigating molecular events involving hMSCs incorporated in a uniquely designed poly(lactic-co-glycolic) acid scaffold, a clinically safe polymer, following inflammatory exposures in a dorsal root ganglion organotypic coculture system...
January 17, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28094009/q-space-myelin-map-imaging-a-new-imaging-technique-for-treatment-evaluation-in-multiple-sclerosis
#20
Jun-Ichi Kira
No abstract text is available yet for this article.
January 4, 2017: Journal of the Neurological Sciences
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