PMID: 9527542Apr 4, 1998Paper

Time course of neuropathology in the spinal cord of G86R superoxide dismutase transgenic mice

The Journal of Comparative Neurology
B M MorrisonJ H Morrison

Abstract

Transgenic mice with a G86R mutation in the mouse superoxide dismutase (SOD-1) gene, which corresponds to a mutation observed in familial amyotrophic lateral sclerosis (ALS), display progressive motor dysfunction leading to paralysis and premature death. In endstage SOD-1 transgenic mice, there is marked loss of spinal motor neurons and interneurons, accumulation of phosphorylated neurofilament inclusions, and reactive astrocytosis. The present study details the time course and ultrastructural appearance of these pathologic changes and correlates the timing of these events with the behavioral symptoms. There is no significant reduction in the number of total neurons, motor neurons, or interneurons in the ventral spinal cord of presymptomatic mice, as compared to age-matched control mice. In contrast, there is a significant reduction in the number of total neurons (-23.5%), motor neurons (-28.9%), and interneurons (-23.5%) in symptomatic SOD-1 transgenic mice. This neuron loss correlates temporally with the onset of reactive astrocytosis and the appearance of phosphorylated neurofilament inclusions. The identical timing of motor neuron and interneuron degeneration in this model of ALS strongly suggests that degeneration in the s...Continue Reading

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Citations

Jul 21, 2004·Proceedings of the National Academy of Sciences of the United States of America·Luc DupuisJean-Philippe Loeffler
Oct 24, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Brigitte van ZundertMark C Bellingham
Oct 27, 2001·Neuropathology and Applied Neurobiology·B StephensR J Guiloff
Aug 16, 2006·Neuro-degenerative Diseases·Luc DupuisJean-Philippe Loeffler
May 14, 2010·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·S M ElBasiounyC J Heckman
May 12, 2009·Neurobiology of Aging·A E KingJ C Vickers
Nov 20, 2015·Experimental Neurology·Anna E KingJames C Vickers
Oct 18, 2008·Experimental Neurology·Diego MinciacchiMarina Bentivoglio
Dec 1, 2015·Neurodegenerative Disease Management·Rosemary ClarkTracey Dickson
Nov 26, 2015·Frontiers in Cellular Neuroscience·Gianfranco NataleFrancesco Fornai
Jul 18, 2009·Muscle & Nerve·Livia PasqualiFrancesco Fornai
Sep 28, 2007·The European Journal of Neuroscience·Eva HedlundOle Isacson
May 15, 2007·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Shintaro HayashiKoichi Okamoto
Oct 22, 2013·Neuron·Christopher J DonnellyJeffrey D Rothstein
Oct 26, 2013·Neuropharmacology·Uri Nimrod Ramírez-JarquínRicardo Tapia
Sep 26, 2003·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·B M KüstJ C V M Copray
Mar 3, 2005·Journal of Neurology·Herbert SchreiberAlbert Christian Ludolph
Jan 9, 2008·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Koji DoiTakao Inoué
Jun 6, 2007·Journal of Neuropathology and Experimental Neurology·Shoichi SasakiMakoto Iwata
Nov 15, 2007·Physiological Genomics·Jose-Luis Gonzalez de AguilarJean-Philippe Loeffler
May 3, 2013·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Daniela MannarelliFrancesco Fattapposta
Oct 25, 2003·Journal of Neuropathology and Experimental Neurology·Susanne PetriJohannes Bufler
Sep 15, 2017·Proceedings of the National Academy of Sciences of the United States of America·Noam D RudnickTom Maniatis
Jun 18, 2009·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Michelle S BalaratnamRoberto J Guiloff

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