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https://www.readbyqxmd.com/read/28429407/spinal-cord-injury-induces-widespread-chronic-changes-in-cerebral-white-matter
#1
Tero Ilvesmäki, Eerika Koskinen, Antti Brander, Teemu Luoto, Juha Öhman, Hannu Eskola
Traumatic spinal cord injuries (SCIs) lead to axonal damage at the trauma site, as well as disconnections within the central nervous system. While the exact mechanisms of the long-term pathophysiological consequences of SCIs are not fully understood, it is known that neuronal damage and degeneration are not limited to the direct proximity of the trauma. Instead, the effects can be detected even in the cerebrum. We examined SCI-induced chronic brain changes with a case-control design using 32 patients and 70 control subjects...
April 21, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28428485/pathological-lesions-in-the-central-nervous-system-and-peripheral-tissues-of-ddy-mice-with-street-rabies-virus-1088-strain
#2
Kazunori Kimitsuki, Kentaro Yamada, Nozomi Shiwa, Satoshi Inoue, Akira Nishizono, Chun-Ho Park
Most studies on rabies virus pathogenesis in animal models have employed fixed rabies viruses, and the results of those employing street rabies viruses have been inconsistent. Therefore, to clarify the pathogenesis of street rabies virus (1088 strain) in mice, 10(6) focus forming units were inoculated into the right hindlimb of ddY mice (6 weeks, female). At 3 days postinoculation (DPI), mild inflammation was observed in the hindlimb muscle. At 5 DPI, ganglion cells in the right lumbosacral spinal dorsal root ganglia showed chromatolysis...
April 20, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28426667/bioenergetic-status-modulates-motor-neuron-vulnerability-and-pathogenesis-in-a-zebrafish-model-of-spinal-muscular-atrophy
#3
Penelope J Boyd, Wen-Yo Tu, Hannah K Shorrock, Ewout J N Groen, Roderick N Carter, Rachael A Powis, Sophie R Thomson, Derek Thomson, Laura C Graham, Anna A L Motyl, Thomas M Wishart, J Robin Highley, Nicholas M Morton, Thomas Becker, Catherina G Becker, Paul R Heath, Thomas H Gillingwater
Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected, with some populations displaying a robust resistance to the disease. Here, we demonstrate that selective vulnerability of distinct motor neuron pools arises from fundamental modifications to their basal molecular profiles...
April 20, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28425503/nerve-degeneration-and-regeneration-in-the-cephalopod-mollusc-octopus-vulgaris-the-case-of-the-pallial-nerve
#4
Pamela Imperadore, Sameer B Shah, Helen P Makarenkova, Graziano Fiorito
Regeneration is a process that restores structure and function of tissues damaged by injury or disease. In mammals complete regeneration is often unsuccessful, while most of the low phyla animals can re-grow many parts of their body after amputation. Cephalopod molluscs, and in particular Octopus vulgaris, are well known for their capacity to regenerate their arms and other body parts, including central and peripheral nervous system. To better understand the mechanism of recovery following nerve injury in this species we investigated the process of axon regrowth and nerve regeneration after complete transection of the Octopus pallial nerves...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28424571/metabolic-vulnerability-in-the-neurodegenerative-disease-glaucoma
#5
REVIEW
Denise M Inman, Mohammad Harun-Or-Rashid
Axons can be several orders of magnitude longer than neural somas, presenting logistical difficulties in cargo trafficking and structural maintenance. Keeping the axon compartment well supplied with energy also presents a considerable challenge; even seemingly subtle modifications of metabolism can result in functional deficits and degeneration. Axons require a great deal of energy, up to 70% of all energy used by a neuron, just to maintain the resting membrane potential. Axonal energy, in the form of ATP, is generated primarily through oxidative phosphorylation in the mitochondria...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28420980/loss-of-melanopsin-expressing-ganglion-cell-subtypes-and-dendritic-degeneration-in-the-aging-human-retina
#6
Gema Esquiva, Pedro Lax, Juan J Pérez-Santonja, José M García-Fernández, Nicolás Cuenca
In mammals, melanopsin-expressing retinal ganglion cells (mRGCs) are, among other things, involved in several non-image-forming visual functions, including light entrainment of circadian rhythms. Considering the profound impact of aging on visual function and ophthalmic diseases, here we evaluate changes in mRGCs throughout the life span in humans. In 24 post-mortem retinas from anonymous human donors aged 10-81 years, we assessed the distribution, number and morphology of mRGCs by immunostaining vertical retinal sections and whole-mount retinas with antibodies against melanopsin...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28416800/axon-degeneration-a-receptor-for-injury
#7
Darran Yates
No abstract text is available yet for this article.
April 18, 2017: Nature Reviews. Neuroscience
https://www.readbyqxmd.com/read/28414271/monitoring-atp-dynamics-in-electrically-active-white-matter-tracts
#8
Andrea Trevisiol, Aiman S Saab, Ulrike Winkler, Grit Marx, Hiromi Imamura, Wiebke Möbius, Kathrin Kusch, Klaus-Armin Nave, Johannes Hirrlinger
In several neurodegenerative diseases and myelin disorders, the degeneration profiles of myelinated axons are compatible with underlying energy deficits. However, it is presently impossible to measure selectively axonal ATP levels in the electrically active nervous system. We combined transgenic expression of an ATP-sensor in neurons of mice with confocal FRET imaging and electrophysiological recordings of acutely isolated optic nerves. This allowed us to monitor dynamic changes and activity-dependent axonal ATP homeostasis at the cellular level and in real time...
April 17, 2017: ELife
https://www.readbyqxmd.com/read/28413156/exposure-of-the-amino-terminus-of-tau-is-a-pathological-event-in-multiple-tauopathies
#9
Benjamin Combs, Nicholas M Kanaan
Pathological changes to the tau protein, including conformational changes and aggregation, are major hallmarks of a group of neurodegenerative disorders known as tauopathies. Among the conformational changes are alterations involving the extreme amino terminus of the protein, known as the phosphatase-activating domain (PAD). Aberrant PAD exposure induces a signaling cascade that leads to disruption of axonal transport, a critical function for neuronal survival. Conformational display of PAD is an early marker of pathological tau in Alzheimer disease (AD), but its role in other tauopathies has yet to be firmly established...
April 13, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28411118/regulation-of-motor-proteins-axonal-transport-deficits-and-adult-onset-neurodegenerative-diseases
#10
REVIEW
Scott T Brady, Gerardo A Morfini
Neurons affected in a wide variety of unrelated adult-onset neurodegenerative diseases (AONDs) typically exhibit a "dying back" pattern of degeneration, which is characterized by early deficits in synaptic function and neuritic pathology long before neuronal cell death. Consistent with this observation, multiple unrelated AONDs including Alzheimer's disease, Parkinson's disease, Huntington's disease, and several motor neuron diseases feature early alterations in kinase-based signaling pathways associated with deficits in axonal transport (AT), a complex cellular process involving multiple intracellular trafficking events powered by microtubule-based motor proteins...
April 11, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28408402/poly-gp-in-cerebrospinal-fluid-links-c9orf72-associated-dipeptide-repeat-expression-to-the-asymptomatic-phase-of-als-ftd
#11
Carina Lehmer, Patrick Oeckl, Jochen H Weishaupt, Alexander E Volk, Janine Diehl-Schmid, Matthias L Schroeter, Martin Lauer, Johannes Kornhuber, Johannes Levin, Klaus Fassbender, Bernhard Landwehrmeyer, Martin H Schludi, Thomas Arzberger, Elisabeth Kremmer, Andrew Flatley, Regina Feederle, Petra Steinacker, Patrick Weydt, Albert C Ludolph, Dieter Edbauer, Markus Otto
The C9orf72 GGGGCC repeat expansion is a major cause of amyotrophic lateral sclerosis and frontotemporal dementia (c9ALS/FTD). Non-conventional repeat translation results in five dipeptide repeat proteins (DPRs), but their clinical utility, overall significance, and temporal course in the pathogenesis of c9ALS/FTD are unclear, although animal models support a gain-of-function mechanism. Here, we established a poly-GP immunoassay from cerebrospinal fluid (CSF) to identify and characterize C9orf72 patients. Significant poly-GP levels were already detectable in asymptomatic C9orf72 mutation carriers compared to healthy controls and patients with other neurodegenerative diseases...
April 13, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28401686/axonal-loss-in-the-multiple-sclerosis-spinal-cord-revisited
#12
N Petrova, D Carassiti, D R Altmann, D Baker, K Schmierer
Preventing chronic disease deterioration is an unmet need in people with multiple sclerosis, where axonal loss is considered a key substrate of disability. Clinically, chronic multiple sclerosis often presents as progressive myelopathy. Spinal cord cross-sectional area assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. However, the association between cross-sectional area and axonal loss, and their correlation with demyelination, have never been systematically investigated using human post mortem tissue...
April 12, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28398512/mutant-spastin-proteins-promote-deficits-in-axonal-transport-through-an-isoform-specific-mechanism-involving-casein-kinase-2-activation
#13
Lanfranco Leo, Carina Weissmann, Matthew Burns, Minsu Kang, Yuyu Song, Liang Qiang, Scott T Brady, Peter W Baas, Gerardo Morfini
Mutations of various genes cause hereditary spastic paraplegia (HSP), a neurological disease involving dying-back degeneration of upper motor neurons. From these, mutations in the SPAST gene encoding the microtubule-severing protein spastin account for most HSP cases. Cumulative genetic and experimental evidence suggests that alterations in various intracellular trafficking events, including fast axonal transport (FAT), may contribute to HSP pathogenesis. However, the mechanisms linking SPAST mutations to such deficits remain largely unknown...
April 7, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28395083/pathological-confirmation-of-optic-neuropathy-in-familial-dysautonomia
#14
Carlos E Mendoza-Santiesteban, Jose-Alberto Palma, Thomas R Hedges, Nora V Laver, Nada Farhat, Lucy Norcliffe-Kaufmann, Horacio Kaufmann
Clinical data suggest that optic neuropathy and retinal ganglion cell loss are the main cause of visual decline in patients with familial dysautonomia, but this has not previously been confirmed by pathological analyses. We studied retinas and optic nerves in 6 eyes from 3 affected patients obtained at autopsy. Analyses included routine neurohistology and immunohistochemistry for neurofilaments, cytochrome c oxidase (COX), and melanopsin-containing ganglion cells. We observed profound axon loss in the temporal portions of optic nerves with relative preservation in the nasal portions; this correlated with clinical and optical coherence tomography findings in 1 patient...
March 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28394203/dysregulation-of-energy-metabolism-in-multiple-sclerosis-measured-in-vivo-with-diffusion-weighted-spectroscopy
#15
Benedetta Bodini, Francesca Branzoli, Emilie Poirion, Daniel García-Lorenzo, Mélanie Didier, Elisabeth Maillart, Julie Socha, Geraldine Bera, Catherine Lubetzki, Itamar Ronen, Stephane Lehericy, Bruno Stankoff
OBJECTIVE: We employed diffusion-weighted magnetic resonance spectroscopy (DW-MRS), which allows to measure in vivo the diffusion properties of metabolites, to explore the functional neuro-axonal damage and the ongoing energetic dysregulation in multiple sclerosis (MS). METHODS: Twenty-five patients with MS and 18 healthy controls (HC) underwent conventional magnetic resonance imaging (MRI) and DW-MRS. The apparent diffusion coefficient (ADC) of total N-acetyl-aspartate (tNAA) and creatine-phosphocreatine (tCr) were measured in the parietal normal-appearing white matter (NAWM) and in the thalamic grey matter (TGM)...
April 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28392448/effect-of-nitric-oxide-to-axonal-degeneration-in-multiple-sclerosis-via-downregulating-monocarboxylate-transporter-1-in-oligodendrocytes
#16
REVIEW
Xiaoyi Tang, Minghong Lan, Mao Zhang, Zhongxiang Yao
Multiple sclerosis (MS) is a neurodegenerative disease of the central nervous system (CNS). Axonal degeneration, one of the main pathological characteristics of MS, is affected by nitric oxide (NO). In turn, NO induces mitochondrial dysfunction of neurons and glial cells. Inadequate glucose causes monocarboxylate transporter 1 (MCT1) to transfer lactate from oligodendrocytes (OLs) to neurons, which decreases MCT1 and results in energy substrate deficit (mainly lactate) in axons. The condition gradually leads to axonal degeneration...
April 6, 2017: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/28389476/defects-in-er-endosome-contacts-impact-lysosome-function-in-hereditary-spastic-paraplegia
#17
Rachel Allison, James R Edgar, Guy Pearson, Tania Rizo, Timothy Newton, Sven Günther, Fiamma Berner, Jennifer Hague, James W Connell, Jürgen Winkler, Jennifer Lippincott-Schwartz, Christian Beetz, Beate Winner, Evan Reid
Contacts between endosomes and the endoplasmic reticulum (ER) promote endosomal tubule fission, but the mechanisms involved and consequences of tubule fission failure are incompletely understood. We found that interaction between the microtubule-severing enzyme spastin and the ESCRT protein IST1 at ER-endosome contacts drives endosomal tubule fission. Failure of fission caused defective sorting of mannose 6-phosphate receptor, with consequently disrupted lysosomal enzyme trafficking and abnormal lysosomal morphology, including in mouse primary neurons and human stem cell-derived neurons...
April 7, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28387380/a-staged-screening-of-registered-drugs-highlights-remyelinating-drug-candidates-for-clinical-trials
#18
C Eleuteri, S Olla, C Veroni, R Umeton, R Mechelli, S Romano, M C Buscarinu, F Ferrari, G Calò, G Ristori, M Salvetti, C Agresti
There is no treatment for the myelin loss in multiple sclerosis, ultimately resulting in the axonal degeneration that leads to the progressive phase of the disease. We established a multi-tiered platform for the sequential screening of drugs that could be repurposed as remyelinating agents. We screened a library of 2,000 compounds (mainly Food and Drug Administration (FDA)-approved compounds and natural products) for cellular metabolic activity on mouse oligodendrocyte precursors (OPC), identifying 42 molecules with significant stimulating effects...
April 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28385104/prolonged-cerebrospinal-fluid-neurofilament-light-chain-increase-in-patients-with-post-traumatic-disorders-of-consciousness
#19
Sergio Bagnato, Luigi M E Grimaldi, Giorgio Di Raimondo, Antonino Sant'Angelo, Cristina Boccagni, Vittorio Virgilio, Maria Andriolo
The mechanisms involved in secondary brain injury after the acute phase of severe traumatic brain injury (TBI) are largely unknown. Ongoing axonal degeneration, consequent to the initial trauma, may lead to secondary brain injury. To test this hypothesis, we evaluated the cerebrospinal fluid (CSF) level of neurofilament light chain (NF-L), a proposed marker of axonal degeneration, in ten patients who developed a severe disorder of consciousness after a TBI, including seven in a minimally conscious state and three with unresponsive wakefulness syndrome (time since brain injury 309 ± 169 days)...
April 7, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28383419/metabolic-changes-in-normal-appearing-white-matter-in-multiple-sclerosis-patients-using-multivoxel-magnetic-resonance-spectroscopy-imaging
#20
Jubao Sun, Hao Song, Yong Yang, Kun Zhang, Xiuju Gao, XiaoPan Li, Li Ni, Pan Lin, Chen Niu
Demyelination and axonal degeneration caused by multiple sclerosis (MS) exist in the white matter and not only in the lesion area. Magnetic resonance spectroscopy (MRS) could provide a unique insight into metabolic changes in the normal appearing white matter (NAWM). To evaluate the subtle axonal degeneration and delineate the spatial distribution of metabolite abnormalities in the NAWM in patients with MS. A total of 17 clinically definite relapsing-remitting MS (RRMS) patients and 21 healthy controls were enrolled in this study...
April 2017: Medicine (Baltimore)
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