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https://www.readbyqxmd.com/read/28322742/potassium-channel-abnormalities-are-consistent-with-early-axon-degeneration-of-motor-axons-in-the-g127x-sod1-mouse-model-of-amyotrophic-lateral-sclerosis
#1
Rikke Maglemose, Anne Hedegaard, Janna Lehnhoff, Kristina Petrova Dimintiyanova, Mihai Moldovan, Lillian Grøndahl, Claire Francesca Meehan
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease, which selectively affects upper and lower motoneurones. The underlying pathophysiology of the disease is complex but electrophysiological studies of peripheral nerves in ALS patients as well as human autopsy studies indicate that a potassium channel dysfunction/loss is present early in the symptomatic phase. It remains unclear to what extent potassium channel abnormalities reflect a specific pathogenic mechanism in ALS. The aim of this study was therefore to investigate the temporal changes in the expression and/or function of potassium channels in motoneurones in the adult G127X SOD1 mouse model of ALS, a model which has a very long presymptomatic phase...
March 16, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28320183/ischemic-optic-neuropathy-as-a-model-of-neurodegenerative-disorder-a-review-of-pathogenic-mechanism-of-axonal-degeneration-and-the-role-of-neuroprotection
#2
REVIEW
Saba Khalilpour, Shahrzad Latifi, Ghazaleh Behnammanesh, Amin Malik Shah Abdul Majid, Aman Shah Abdul Majid, Ali Tamayol
Optic neuropathy is a neurodegenerative disease which involves optic nerve injury. It is caused by acute or intermittent insults leading to visual dysfunction. There are number of factors, responsible for optic neuropathy, and the optic nerve axon is affected in all type which causes the loss of retinal ganglion cells. In this review we will highlight various mechanisms involved in the cell loss cascades during axonal degeneration as well as ischemic optic neuropathy. These mechanisms include oxidative stress, excitotoxicity, angiogenesis, neuroinflammation and apoptosis following retinal ischemia...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28315275/sigma-1-receptor-in-motoneuron-disease
#3
Renzo Mancuso, Xavier Navarro
Amyotrophic Lateral Sclerosis (ALS ) is a neurodegenerative disease affecting spinal cord and brain motoneurons , leading to paralysis and early death. Multiple etiopathogenic mechanisms appear to contribute in the development of ALS , including glutamate excitotoxicity, oxidative stress , protein misfolding, mitochondrial defects, impaired axonal transport, inflammation and glial cell alterations. The Sigma-1 receptor is highly expressed in motoneurons of the spinal cord, particularly enriched in the endoplasmic reticulum (ER) at postsynaptic cisternae of cholinergic C-terminals...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28303068/high-dose-intravenous-pulse-steroid-therapy-for-optic-disc-swelling-and-subretinal-fluid-in-non-arteritic-anterior-ischemic-optic-neuropathy
#4
Kei Takayama, Hiroki Kaneko, Shu Kachi, Eimei Ra, Yasuki Ito, Hiroko Terasaki
Non-arteritic anterior ischemic optic neuropathy (NAION) is a disease with microvascular abnormality that causes acute optic disc swelling (ODS) and, in severe cases, subretinal fluid (SRF) accumulation. ODS causes compartment syndrome and subsequent axonal degeneration and loss of retinal ganglion cells by apoptosis. No treatment modalities have been effective, although some cases improved after the intake of oral systemic steroids. We reported a case of a 72-year-old man who was referred due to a visual defect in the right eye...
February 2017: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28302146/relationship-of-acute-axonal-damage-wallerian-degeneration-and-clinical-disability-in-multiple-sclerosis
#5
Shailender Singh, Tobias Dallenga, Anne Winkler, Shanu Roemer, Brigitte Maruschak, Heike Siebert, Wolfgang Brück, Christine Stadelmann
BACKGROUND: Axonal damage and loss substantially contribute to the incremental accumulation of clinical disability in progressive multiple sclerosis. Here, we assessed the amount of Wallerian degeneration in brain tissue of multiple sclerosis patients in relation to demyelinating lesion activity and asked whether a transient blockade of Wallerian degeneration decreases axonal loss and clinical disability in a mouse model of inflammatory demyelination. METHODS: Wallerian degeneration and acute axonal damage were determined immunohistochemically in the periplaque white matter of multiple sclerosis patients with early actively demyelinating lesions, chronic active lesions, and inactive lesions...
March 17, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28300211/als-along-the-axons-expression-of-coding-and-noncoding-rna-differs-in-axons-of-als-models
#6
Nimrod Rotem, Iddo Magen, Ariel Ionescu, Noga Gershoni-Emek, Topaz Altman, Christopher J Costa, Tal Gradus, Metsada Pasmanik-Chor, Dianna E Willis, Iddo Z Ben-Dov, Eran Hornstein, Eran Perlson
Amyotrophic lateral sclerosis (ALS) is a multifactorial lethal motor neuron disease with no known treatment. Although the basic mechanism of its degenerative pathogenesis remains poorly understood, a subcellular spatial alteration in RNA metabolism is thought to play a key role. The nature of these RNAs remains elusive, and a comprehensive characterization of the axonal RNAs involved in maintaining neuronal health has yet to be described. Here, using cultured spinal cord (SC) neurons grown using a compartmented platform followed by next-generation sequencing (NGS) technology, we find that RNA expression differs between the somatic and axonal compartments of the neuron, for both mRNA and microRNA (miRNA)...
March 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28299359/aifm1-mutation-presenting-with-fatal-encephalomyopathy-and-mitochondrial-disease-in-an-infant
#7
Sarah U Morton, Sanjay P Prabhu, Hart G W Lidov, Jiahai Shi, Irina Anselm, Catherine A Brownstein, Matthew N Bainbridge, Alan H Beggs, Sara O Vargas, Pankaj B Agrawal
Apoptosis-inducing factor mitochondrion-associated 1 (AIFM1), encoded by the gene AIFM1, has roles in electron transport, apoptosis, ferredoxin metabolism, reactive oxygen species generation, and immune system regulation. Here we describe a patient with a novel AIFM1 variant presenting unusually early in life with mitochondrial disease, rapid deterioration, and death. Autopsy, at the age of 4 mo, revealed features of mitochondrial encephalopathy, myopathy, and involvement of peripheral nerves with axonal degeneration...
March 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28298170/concussion-induces-hippocampal-circuitry-disruption-in-swine
#8
John A Wolf, Brian N Johnson, Victoria E Johnson, Mary E Putt, Kevin D Browne, Constance J Mietus, Daniel P Brown, Kathryn L Wofford, Douglas H Smith, M Sean Grady, Akiva S Cohen, D Kacy Cullen
Hippocampal-dependent deficits in learning and memory formation are a prominent feature of traumatic brain injury (TBI), however the role of the hippocampus in cognitive dysfunction after concussion (mild TBI) is unknown. We therefore investigated functional and structural changes in the swine hippocampus following TBI using a model of head rotational acceleration that closely replicates the biomechanics and neuropathology of closed-head TBI in humans. We examined neurophysiological changes using a novel ex vivo hippocampal slice paradigm with extracellular stimulation and recording in the dentate gyrus and CA1 occurring at 7 days following non-impact inertial TBI in swine...
March 16, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28292833/mesoscale-architecture-shapes-initiation-and-richness-of-spontaneous-network-activity
#9
Samora Okujeni, Steffen Kandler, Ulrich Egert
Spontaneous activity in the absence of external input, including propagating waves of activity, is a robust feature of neuronal networks in vivo and in vitro. The neurophysiological and anatomical requirements for initiation and persistence of such activity, however, are poorly understood, as is their role in the function of neuronal networks. Computational network studies indicate that clustered connectivity may foster the generation, maintenance and richness of spontaneous activity. Since this mesoscale architecture cannot be systematically modified in intact tissue, testing these predictions is impracticable in vivo...
March 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28285993/death-receptor-6-promotes-wallerian-degeneration-in-peripheral-axons
#10
Kanchana K Gamage, Irene Cheng, Rachel E Park, Mardeen S Karim, Kazusa Edamura, Christopher Hughes, Anthony J Spano, Alev Erisir, Christopher D Deppmann
Axon degeneration during development is required to sculpt a functional nervous system and is also a hallmark of pathological insult, such as injury [1, 2]. Despite similar morphological characteristics, very little overlap in molecular mechanisms has been reported between pathological and developmental degeneration [3-5]. In the peripheral nervous system (PNS), developmental axon pruning relies on receptor-mediated extrinsic degeneration mechanisms to determine which axons are maintained or degenerated [5-7]...
March 20, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28283581/mtcl1-plays-an-essential-role-in-maintaining-purkinje-neuron-axon-initial-segment
#11
Tomoko Satake, Kazunari Yamashita, Kenji Hayashi, Satoko Miyatake, Miwa Tamura-Nakano, Hiroshi Doi, Yasuhide Furuta, Go Shioi, Eriko Miura, Yukari H Takeo, Kunihiro Yoshida, Hiroyuki Yahikozawa, Naomichi Matsumoto, Michisuke Yuzaki, Atsushi Suzuki
The axon initial segment (AIS) is a specialized domain essential for neuronal function, the formation of which begins with localization of an ankyrin-G (AnkG) scaffold. However, the mechanism directing and maintaining AnkG localization is largely unknown. In this study, we demonstrate that in vivo knockdown of microtubule cross-linking factor 1 (MTCL1) in cerebellar Purkinje cells causes loss of axonal polarity coupled with AnkG mislocalization. MTCL1 lacking MT-stabilizing activity failed to restore these defects, and stable MT bundles spanning the AIS were disorganized in knockdown cells...
March 10, 2017: EMBO Journal
https://www.readbyqxmd.com/read/28283540/extrinsic-repair-of-injured-dendrites-as-a-paradigm-for-regeneration-by-fusion-in-caenorhabditis-elegans
#12
Meital Oren-Suissa, Tamar Gattegno, Veronika Kravtsov, Benjamin Podbilewicz
Injury triggers regeneration of axons and dendrites. Research identified factors required for axonal regeneration outside the CNS, but little is known about regeneration triggered by dendrotomy. Here we study neuronal plasticity triggered by dendrotomy and determine the fate of complex PVD arbors following laser surgery of dendrites. We find that severed primary dendrites grow towards each other and reconnect via branch fusion. Simultaneously, terminal branches lose self-avoidance and grow towards each other, meeting and fusing at the tips via an AFF-1-mediated process...
March 10, 2017: Genetics
https://www.readbyqxmd.com/read/28283336/tissue-engineering-with-peripheral-blood-derived-mesenchymal-stem-cells-promotes-the-regeneration-of-injured-peripheral-nerves
#13
Mengjie Pan, Xianghai Wang, Yijing Chen, Shangtao Cao, Jinkun Wen, Guofeng Wu, Yuanyuan Li, Lixia Li, Changhui Qian, Zhenqi Qin, Zhenlin Li, Dandan Tan, Zhihao Fan, Wutian Wu, Jiasong Guo
Peripheral nerve injury repair can be enhanced by Schwann cell (SC) transplantation, but clinical applications are limited by the lack of a cell source. Thus, alternative systems for generating SCs are desired. Herein, we found the peripheral blood-derived mesenchymal stem cells (PBMSCs) could be induced into SC like cells with expressing SC-specific markers (S100, P75NTR and CNPase) and functional factors (NGF, NT-3, c-Fos, and Krox20). When the induced PBMSCs (iPBMSCs) were transplanted into crushed rat sciatic nerves, they functioned as SCs by wrapping the injured axons and expressing myelin specific marker of MBP...
March 7, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28275163/axonal-degeneration-in-retinal-ganglion-cells-is-associated-with-a-membrane-polarity-sensitive-redox-process
#14
Mohammadali Almasieh, Maria-Magdalena Catrinescu, Loïc Binan, Santiago Costantino, Leonard A Levin
Axonal degeneration is a pathophysiological mechanism common to several neurodegenerative diseases. The slow Wallerian degeneration (Wld(S)) mutation, which results in reduced axonal degeneration in the central and peripheral nervous systems, has provided insight for a redox-dependent mechanism by which axons undergo self-destruction. We studied early molecular events in axonal degeneration with single-axon laser axotomy and time-lapse imaging, monitoring the initial changes in transected axons of purified retinal ganglion cells (RGCs) from wild-type and Wld(S) rat retinas using a polarity-sensitive annexin-based biosensor (annexin B12-Cys101,Cys260-N,N'-dimethyl-N-(iodoacetyl)-N'-(7-nitrobenz-2-oxa-1,3-diazol-4-yl) ethylenediamine)...
March 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28273913/als-linked-fus-exerts-a-gain-of-toxic-function-involving-aberrant-p38-mapk-activation
#15
Reddy Ranjith K Sama, Claudia Fallini, Rodolfo Gatto, Jeanne E McKeon, Yuyu Song, Melissa S Rotunno, Saul Penaranda, Izrail Abdurakhmanov, John E Landers, Gerardo Morfini, Scott T Brady, Daryl A Bosco
Mutations in Fused in Sarcoma/Translocated in Liposarcoma (FUS) cause familial forms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by progressive axonal degeneration mainly affecting motor neurons. Evidence from transgenic mouse models suggests mutant forms of FUS exert an unknown gain-of-toxic function in motor neurons, but mechanisms underlying this effect remain unknown. Towards this end, we studied the effect of wild type FUS (FUS WT) and three ALS-linked variants (G230C, R521G and R495X) on fast axonal transport (FAT), a cellular process critical for appropriate maintenance of axonal connectivity...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28270908/targeting-oxidative-stress-for-treatment-of-glaucoma-and-optic-neuritis
#16
REVIEW
Atsuko Kimura, Kazuhiko Namekata, Xiaoli Guo, Takahiko Noro, Chikako Harada, Takayuki Harada
Glaucoma is a neurodegenerative disease of the eye and it is one of the leading causes of blindness. Glaucoma is characterized by progressive degeneration of retinal ganglion cells (RGCs) and their axons, namely, the optic nerve, usually associated with elevated intraocular pressure (IOP). Current glaucoma therapies target reduction of IOP, but since RGC death is the cause of irreversible vision loss, neuroprotection may be an effective strategy for glaucoma treatment. One of the risk factors for glaucoma is increased oxidative stress, and drugs with antioxidative properties including valproic acid and spermidine, as well as inhibition of apoptosis signal-regulating kinase 1, an enzyme that is involved in oxidative stress, have been reported to prevent glaucomatous retinal degeneration in mouse models of glaucoma...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28270748/huntingtin-is-required-for-neural-but-not-cardiac-pancreatic-progenitor-differentiation-of-mouse-embryonic-stem-cells-in-vitro
#17
Man Shan Yu, Naoko Tanese
Mutation in the huntingtin (HTT) gene causes Huntington's disease (HD). It is an autosomal dominant trinucleotide-repeat expansion disease in which CAG repeat sequence expands to >35. This results in the production of mutant HTT protein with an increased stretch of glutamines near the N-terminus. The wild type HTT gene encodes a 350 kD protein whose function remains elusive. Mutant HTT protein has been implicated in transcription, axonal transport, cytoskeletal structure/function, signal transduction, and autophagy...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28265751/peripheral-nerve-diffusion-tensor-imaging-as-a-measure-of-disease-progression-in-als
#18
Neil G Simon, Jim Lagopoulos, Sita Paling, Casey Pfluger, Susanna B Park, James Howells, Thomas Gallagher, Michel Kliot, Robert D Henderson, Steve Vucic, Matthew C Kiernan
Clinical trial design in amyotrophic lateral sclerosis (ALS) remains hampered by a lack of reliable and sensitive biomarkers of disease progression. The present study evaluated peripheral nerve diffusion tensor imaging (DTI) as a surrogate marker of axonal degeneration in ALS. Longitudinal studies were undertaken in 21 ALS patients studied at 0 and 3 months, and 19 patients at 0, 3 and 6 months, with results compared to 13 age-matched controls. Imaging metrics were correlated across a range of functional assessments including amyotrophic lateral sclerosis functional rating scale revised (ALSFRS-R), lower limb muscle strength (Medical Research Council sum score, MRCSS-LL), compound muscle action potential amplitudes and motor unit number estimation (MUNE)...
March 6, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28265351/elevated-axonal-membrane-permeability-and-its-correlation-with-motor-deficits-in-an-animal-model-of-multiple-sclerosis
#19
Gary Leung, Melissa Tully, Jonathan Tang, Shengxi Wu, Riyi Shi
BACKGROUND: It is increasingly clear that in addition to myelin disruption, axonal degeneration may also represent a key pathology in multiple sclerosis (MS). Hence, elucidating the mechanisms of axonal degeneration may not only enhance our understanding of the overall MS pathology, but also elucidate additional therapeutic targets. The objective of this study is assess the degree of axonal membrane disruption and its significance in motor deficits in EAE mice. METHODS: Experimental Autoimmune Encephalomyelitis was induced in mice by subcutaneous injection of myelin oligodendrocyte glycoprotein/complete Freud's adjuvant emulsion, followed by two intraperitoneal injections of pertussis toxin...
2017: Translational Neurodegeneration
https://www.readbyqxmd.com/read/28262487/nmn-deamidase-delays-wallerian-degeneration-and-rescues-axonal-defects-caused-by-nmnat2-deficiency-in%C3%A2-vivo
#20
Michele Di Stefano, Andrea Loreto, Giuseppe Orsomando, Valerio Mori, Federica Zamporlini, Richard P Hulse, Jamie Webster, Lucy F Donaldson, Martin Gering, Nadia Raffaelli, Michael P Coleman, Jonathan Gilley, Laura Conforti
Axons require the axonal NAD-synthesizing enzyme NMNAT2 to survive. Injury or genetically induced depletion of NMNAT2 triggers axonal degeneration or defective axon growth. We have previously proposed that axonal NMNAT2 primarily promotes axon survival by maintaining low levels of its substrate NMN rather than generating NAD; however, this is still debated. NMN deamidase, a bacterial enzyme, shares NMN-consuming activity with NMNAT2, but not NAD-synthesizing activity, and it delays axon degeneration in primary neuronal cultures...
March 20, 2017: Current Biology: CB
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