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axon degeneration

Ramón Martínez-Mármol, Bruna Barneda-Zahonero, David Soto, Rosa Maria Andrés, Elena Coccia, Xavier Gasull, Laura Planells-Ferrer, Rana S Moubarak, Eduardo Soriano, Joan X Comella
Caspases have recently emerged as key regulators of axonal pruning and degeneration and of long-term depression (LTD), a long-lasting form of synaptic plasticity. However, the mechanism underlying these functions remains unclear. In this context, XIAP has been shown to modulate these processes. The neuron-specific form of FAIM protein (FAIM-L) is a death receptor antagonist that stabilizes XIAP protein levels, thus preventing death receptor-induced neuronal apoptosis. Here we show that FAIM-L modulates synaptic transmission, prevents chemical-LTD induction in hippocampal neurons, and thwarts axon degeneration after nerve growth factor (NGF) withdrawal...
October 21, 2016: Scientific Reports
Charanjit Kaur, Eng-Ang Ling
Transitory cavities associated with the ventricular system represent probably one of the most unique features in the developing mammalian brain. In rodents, the cavities exist transiently in the developing brain and do not appear to be associated with any pathological events. Among the various cavities, the pyramidal-shaped cavum septum pellucidum (CSP) located beneath the corpus callosum and between the lateral ventricles is most well defined. In addition to the CSP are the bilateral subependymal cysts that are consistently associated with the third and fourth ventricles as well as the aqueduct...
October 20, 2016: Journal of Anatomy
Katherine Zukor, Hong Wang, Brett L Hurst, Venkatraman Siddharthan, Arnaud Van Wettere, Paul M Pilowsky, John D Morrey
Neurological respiratory deficits are serious outcomes of West Nile virus (WNV) disease. WNV patients requiring intubation have a poor prognosis. We previously reported that WNV-infected rodents also appear to have respiratory deficits when assessed by whole-body plethysmography and diaphragmatic electromyography. The purpose of this study was to determine if the nature of the respiratory deficits in WNV-infected rodents is neurological and if deficits are due to a disorder of brainstem respiratory centers, cervical spinal cord (CSC) phrenic motor neuron (PMN) circuitry, or both...
October 19, 2016: Journal of Neurovirology
George Taylor-Walker, Savannah A Lynn, Eloise Keeling, Rosie Munday, David A Johnston, Anton Page, Jennifer A Scott, Srini Goverdhan, Andrew J Lotery, J Arjuna Ratnayaka
Age-related Macular Degeneration (AMD) is a common, irreversible blinding condition that leads to the loss of central vision. AMD has a complex aetiology with both genetic as well as environmental risks factors, and share many similarities with Alzheimer's disease. Recent findings have contributed significantly to unravelling its genetic architecture that is yet to be matched by molecular insights. Studies are made more challenging by observations that aged and AMD retinas accumulate the highly pathogenic Alzheimer's-related Amyloid beta (Aβ) group of peptides, for which there appears to be no clear genetic basis...
October 14, 2016: Experimental Eye Research
Mansi R Khanna, Jane Kovalevich, Virginia M-Y Lee, John Q Trojanowski, Kurt R Brunden
A group of neurodegenerative diseases referred to as tauopathies are characterized by the presence of brain cells harboring inclusions of pathological species of the tau protein. These disorders include Alzheimer's disease and frontotemporal lobar degeneration due to tau pathology, including progressive supranuclear palsy, corticobasal degeneration, and Pick's disease. Tau is normally a microtubule (MT)-associated protein that appears to play an important role in ensuring proper axonal transport, but in tauopathies tau becomes hyperphosphorylated and disengages from MTs, with consequent misfolding and deposition into inclusions that mainly affect neurons but also glia...
October 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
Abdurrahman Emir, Yıldıray Kalkan, Habib Bostan
OBJECTIVES: We investigated the histopathological effects of metamizole sodium (MS) on the sciatic nerve. MATERIALS AND METHODS: This study was performed using 48 adult male Wistar albino rats. Ten groups were constituted with 6 rats in each group. MS injection into the sciatic nerve (group 1), MS injection into the muscle [group 3 (50 mg/kg, 0.4 ml) and group 5 (50 mg/kg, 0.8 ml)], MS injection into the muscle cavity in the vicinity of the sciatic nerve [group 2 (50 mg/kg, 0...
August 2016: Iranian Journal of Basic Medical Sciences
Yu Kobayashi, Jun Tohyama, Tomoyuki Akiyama, Shinichi Magara, Hideshi Kawashima, Noriyuki Akasaka, Mitsuko Nakashima, Hirotomo Saitsu, Naomichi Matsumoto
Cerebral folate deficiency due to folate receptor 1 gene (FOLR1) mutations is an autosomal recessive disorder resulting from a brain-specific folate transport defect. It is characterized by late infantile onset, severe psychomotor regression, epilepsy, and leukodystrophy. We describe a consanguineous girl exhibiting severe developmental regression, intractable epilepsy, polyneuropathy, and profound hypomyelination with cortical involvement. Magnetic resonance imaging showed cortical disturbances in addition to profound hypomyelination and cerebellar atrophy...
October 12, 2016: Brain & Development
A Spallone, M Çelniku
A 49 years-old man came to our attention with severe flaccid paraparesis occurring 10 days before, almost immediately after he had immerged his legs in boiling water for treating his severe left lombo-crural pain. This was known to be due to a right L3-L4 herniated disk diagnosed by MRI. At the examination he showed severe motor paresis and absent reflexes of his lower limbs, whilst crural pain was very mild and sensation and urinary function were unaffected. The results of his neurological examination led us to suspect an acute motor axon degeneration related to the heating shock...
October 11, 2016: World Neurosurgery
Carla Brancia, Barbara Noli, Marina Boido, Andrea Boi, Roberta Puddu, Giuseppe Borghero, Francesco Marrosu, Paolo Bongioanni, Sandro Orrù, Barbara Manconi, Filomena D'Amato, Irene Messana, Federica Vincenzoni, Alessandro Vercelli, Gian-Luca Ferri, Cristina Cocco
VGF mRNA is widely expressed in areas of the nervous system known to degenerate in Amyotrophic Lateral Sclerosis (ALS), including cerebral cortex, brainstem and spinal cord. Despite certain VGF alterations are reported in animal models, little information is available with respect to the ALS patients. We addressed VGF peptide changes in fibroblast cell cultures and in plasma obtained from ALS patients, in parallel with spinal cord and plasma samples from the G93A-SOD1 mouse model. Antisera specific for the C-terminal end of the human and mouse VGF proteins, respectively, were used in immunohistochemistry and enzyme-linked immunosorbent assay (ELISA), while gel chromatography and HPLC/ESI-MS/MS were used to identify the VGF peptides present...
2016: PloS One
Yo Sasaki, Takashi Nakagawa, Xianrong Mao, Aaron DiAntonio, Jeffrey Milbrandt
Overexpression of the NAD(+) biosynthetic enzyme NMNAT1 leads to preservation of injured axons. While increased NAD(+) or decreased NMN levels are thought to be critical to this process, the mechanism(s) of this axon protection remain obscure. Using steady-state and flux analysis of NAD(+) metabolites in healthy and injured mouse dorsal root ganglion axons, we find that rather than altering NAD(+) synthesis, NMNAT1 instead blocks the injury-induced, SARM1-dependent NAD(+) consumption that is central to axon degeneration...
October 13, 2016: ELife
Marco Bacigaluppi, Gianluca Luigi Russo, Luca Peruzzotti-Jametti, Silvia Rossi, Stefano Sandrone, Erica Butti, Roberta De Ceglia, Andrea Bergamaschi, Caterina Motta, Mattia Gallizioli, Valeria Studer, Emanuela Colombo, Cinthia Farina, Giancarlo Comi, Letterio Salvatore Politi, Luca Muzio, Claudia Villani, Roberto William Invernizzi, Dirk Matthias Hermann, Diego Centonze, Gianvito Martino
: Ischemic stroke is the leading cause of disability, but effective therapies are currently widely lacking. Recovery from stroke is very much dependent on the possibility to develop treatments able to both halt the neurodegenerative process as well as to foster adaptive tissue plasticity. Here we show that ischemic mice treated with neural precursor cell (NPC) transplantation had on neurophysiological analysis, early after treatment, reduced presynaptic release of glutamate within the ipsilesional corticospinal tract (CST), and an enhanced NMDA-mediated excitatory transmission in the contralesional CST...
October 12, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Yuya Yamagishi, Marc Tessier-Lavigne
Axon degeneration is a tightly regulated, self-destructive program that is a critical feature of many neurodegenerative diseases, but the molecular mechanisms regulating this program remain poorly understood. Here, we identify S-phase kinase-associated protein 1A (Skp1a), a core component of a Skp/Cullin/F-box (SCF)-type E3 ubiquitin ligase complex, as a critical regulator of axon degeneration after injury in mammalian neurons. Depletion of Skp1a prolongs survival of injured axons in vitro and in the optic nerve in vivo...
October 11, 2016: Cell Reports
Ibrahim Ali Ozemir, Ferman Ozyalvac, Gorkem Yildiz, Tunc Eren, Zeynep Aydin-Ozemir, Orhan Alimoglu
BACKGROUND: Diabetes mellitus may cause degeneration in the myelin and/or axonal structures of peripheral nerves. The aim of this study was to investigate the effects of diabetic neuropathy on intraoperative neuromonitoring findings such as latency and amplitude values of the recurrent laryngeal nerves during thyroidectomy. To our knowledge this is the first study to report comparison of the electrophysiologic features of diabetic and non-diabetic patients. MATERIALS AND METHODS: One-hundred-and-eleven consecutive patients who received neuromonitoring during thyroidectomy between 2013 and 2015 were included to study...
October 5, 2016: International Journal of Surgery
E N Barker, L J Dawson, J H Rose, S Van Meervenne, O Frykman, C Rohdin, A Leijon, K E Soerensen, J Järnegren, G C Johnson, D P O'Brien, N Granger
BACKGROUND: Neurodegenerative diseases are a heterogeneous group of disorders characterized by loss of neurons and are commonly associated with a genetic mutation. HYPOTHESIS/OBJECTIVES: To characterize the clinical and histopathological features of a novel degenerative neurological disease affecting the brain of young adult Nova Scotia Duck Tolling Retrievers (NSDTRs). ANIMALS: Nine, young adult, related NSDTRs were evaluated for neurological dysfunction and rapid eye movement sleep behavior disorder...
September 2016: Journal of Veterinary Internal Medicine
Andrew R Schwendeman, Shai Shaham
Programmed cell death is a ubiquitous process in metazoan development. Apoptosis, one cell death form, has been studied extensively. However, mutations inactivating key mammalian apoptosis regulators do not block most developmental cell culling, suggesting that other cell death pathways are likely important. Recent work in the nematode Caenorhabditis elegans identified a non-apoptotic cell death form mediating the demise of the male-specific linker cell. This cell death process (LCD, linker cell-type death) is morphologically conserved, and its molecular effectors also mediate axon degeneration in mammals and Drosophila...
2016: PloS One
Satoshi Sugita, Leland L Fleming, Caleb Wood, Sydney K Vaughan, Matheus P S M Gomes, Wallace Camargo, Ligia A Naves, Vania F Prado, Marco A M Prado, Cristina Guatimosim, Gregorio Valdez
BACKGROUND: Cholinergic dysfunction occurs during aging and in a variety of diseases, including amyotrophic lateral sclerosis (ALS). However, it remains unknown whether changes in cholinergic transmission contributes to age- and disease-related degeneration of the motor system. Here we investigated the effect of moderately increasing levels of synaptic acetylcholine (ACh) on the neuromuscular junction (NMJ), muscle fibers, and motor neurons during development and aging and in a mouse model for amyotrophic lateral sclerosis (ALS)...
2016: Skeletal Muscle
Pooja Teotia, Divyan A Chopra, Shashank Manohar Dravid, Matthew J Van Hook, Fang Qiu, John Morrison, Angie Rizzino, Iqbal Ahmad
Glaucoma is a complex group of diseases wherein a selective degeneration of retinal ganglion cells (RGCs) leads to irreversible loss of vision. A comprehensive approach to glaucomatous RGC degeneration may include stem cells to functionally replace dead neurons through transplantation and understand RGCs vulnerability using a disease in a dish stem cell model. Both approaches require the directed generation of stable, functional, and target-specific RGCs from renewable sources of cells, i.e., the embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs)...
October 6, 2016: Stem Cells
Alessandra Bua, Melania Ruggeri, Stefania Zanetti, Paola Molicotti
Multiple sclerosis is a chronic inflammatory disease of the central nervous system characterized by damage to myelin and axons, over time leading to progressive neuronal degeneration and microglial activation. There is still no curative treatment, but during the last 20 years eight different therapies have become available including interferon beta, glatiramer acetate, teriflunomide, dimethyl fumarate, natalizumab, fingolimod, alemtuzumab, mitoxantrone and teriflunomide. Teriflunomide is an immunomodulatory drug that exerts an inhibitory effect on T cell activation in central nervous system of the patients with multiple sclerosis...
October 4, 2016: Medical Microbiology and Immunology
Pravin Salunke, Karthigeyan Madhivanan, Nasib Kamali, Ravi Garg
Bilateral abducens and facial palsy following head injury are extremely rare. We present a patient with post-traumatic bilateral facial and abducens palsy. There were bitemporal fractures that did not correspond with the facial canal. Despite complete facial palsy with axonal degeneration and > 90% facial muscle degenervation, conservative management helped. This report highlights the importance of conservative management in post-traumatic complete facial palsy especially when the fracture line does not correspond with the facial canal...
October 2016: Asian Journal of Neurosurgery
Víctor Manuel Asensio-Sánchez, Javier Foncubierta
INTRODUCTION: Most pituitary adenomas are clinically inactive. In patients with long-standing compression of the optic chiasm, ganglion cells may undergo axonal degeneration. Spectral domain optical coherence tomography (SD-OCT) is able to identify retinal nerve fiber layer (RNFL) and ganglion cell loss in the retina. We present a case in which SD-OCT was used to diagnose an asymptomatic pituitary macroadenoma. CLINICAL CASE: A 48-year-old female presented with progressive vision loss in both eyes...
2016: International Medical Case Reports Journal
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