Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies.

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Felix GeserJohn Q Trojanowski

Abstract

It is now established that pathological transactive response DNA-binding protein with a Mr of 43 kD (TDP-43) on sodium dodecyl sulfate-polyacrylamide gel electrophoresis is the major disease protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with ubiquitin-positive inclusions (now known as FTLD-TDP). In fact, the discovery of pathological TDP-43 solidified the idea that these disorders are multi-system diseases and this led to the concept of a TDP-43 proteinopathy as a spectrum of disorders comprised of different clinical and pathological entities extending from ALS to ALS with cognitive impairment/dementia and FTLD-TDP without or with motor neuron disease (FTLD-MND). These align along a broad disease continuum sharing similar pathogenetic mechanisms linked to pathological TDP-43. We here review salient findings in the development of a concept of TDP-43 proteinopathy as a novel group of neurodegenerative diseases similar in concept to alpha-synucleinopathies and tauopathies.

References

Jun 1, 1970·Journal of Neurology, Neurosurgery, and Psychiatry·B BrownellJ T Hughes
Oct 1, 1996·Neurology·A Hirano
Jul 24, 2001·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·B R BrooksUNKNOWN World Federation of Neurology Research Group on Motor Neuron Diseases
Nov 16, 2001·Archives of Neurology·G M McKhannUNKNOWN Work Group on Frontotemporal Dementia and Pick's Disease
Oct 4, 2002·Proceedings of the National Academy of Sciences of the United States of America·I-Fan WangC-K James Shen
Apr 24, 2004·American Journal of Human Genetics·Ying-Zhang ChenPhillip F Chance
Nov 9, 2004·Nature·Richard I GregoryRamin Shiekhattar
May 7, 2005·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Richard A ArmstrongNigel J Cairns
Jul 22, 2005·Brain : a Journal of Neurology·Andrew KerteszDavid G Munoz
Oct 22, 2005·Lancet Neurology·David NearyDavid Mann
Oct 29, 2005·Nucleic Acids Research·Pablo Arrisi MercadoFrancisco E Baralle
May 24, 2006·Annals of Neurology·Mark S FormanMurray Grossman
Apr 17, 2007·Neuroscience Letters·Sara RollinsonStuart M Pickering-Brown
May 8, 2007·Molecular and Cellular Neurosciences·Michael J StrongChristen Shoesmith
Jun 5, 2007·Neuroscience Letters·Janice RobertsonMichael J Strong
Jun 15, 2007·Acta Neuropathologica·Dennis W DicksonCatalina Amador-Ortiz
Jun 21, 2007·Acta Neuropathologica·Nigel J CairnsUNKNOWN Consortium for Frontotemporal Lobar Degeneration
Jun 27, 2007·The American Journal of Pathology·Nigel J CairnsIan R A Mackenzie
Jul 7, 2007·Neurobiology of Aging·Axel SchumacherMatthias Riemenschneider
Nov 6, 2007·Frontiers in Bioscience : a Journal and Virtual Library·Emanuele Buratti, Francisco E Baralle
Nov 16, 2007·Acta Neuropathologica·Nicholas J BrandmeirJohn Q Trojanowski
Dec 11, 2007·Neurobiology of Aging·Ilse GijselinckMarc Cruts
Dec 20, 2007·Journal of Neuropathology and Experimental Neurology·Teresa SanelliJanice Robertson
Jan 11, 2008·Neurology·K SleegersC Van Broeckhoven
Jan 29, 2008·Human Molecular Genetics·Alice S Chen-PlotkinVirginia M-Y Lee
Feb 22, 2008·Annals of Neurology·Michael A GitchoNigel J Cairns
Feb 29, 2008·Proceedings of the National Academy of Sciences of the United States of America·Youhna M AyalaFrancisco E Baralle

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Citations

Jan 3, 2012·Acta Neuropathologica·Johannes BrettschneiderJohn Q Trojanowski
Jan 7, 2011·Journal of Neural Transmission·Tuomas RauramaaIrina Alafuzoff
Oct 19, 2010·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Maria Teresa GiordanaAnna Montuschi
Aug 4, 2011·Journal of Molecular Neuroscience : MN·Tania F Gendron, Leonard Petrucelli
Dec 1, 2011·Nature Reviews. Neuroscience·Edward B LeeJohn Q Trojanowski
Nov 13, 2013·Nature Reviews. Neurology·Heiko BraakKelly Del Tredici
Jan 19, 2012·Brain : a Journal of Neurology·Azusa UchidaTakanori Yokota
Nov 22, 2011·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Takayuki KosakaHitoshi Takahashi
Nov 3, 2012·Journal of Neurology, Neurosurgery, and Psychiatry·David J IrwinMurray Grossman
Jan 29, 2011·Journal of Biomedicine & Biotechnology·L Van Den Bosch
Jan 6, 2011·The Journal of Clinical Investigation·Lionel M IgazVirginia M-Y Lee
Jun 16, 2012·Molecular Neurodegeneration·Zuo-Shang Xu
Jun 16, 2011·PloS One·Meng-Jau LinC-K James Shen
Dec 3, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Nicole F LiachkoBrian C Kraemer
Sep 7, 2012·CNS Drugs·Rachel E Seltman, Brandy R Matthews
Mar 19, 2014·Nature Reviews. Neurology·V Hugh Perry, Clive Holmes
Feb 22, 2013·Human Molecular Genetics·Channa A A HewamaddumaPamela J Shaw
Jun 19, 2012·Progress in Neuro-psychopharmacology & Biological Psychiatry·Junxia X Tang, Maryellen F Eckenhoff
Nov 6, 2012·Acta Neuropathologica·John L RobinsonJohn Q Trojanowski
Mar 20, 2012·Acta Neuropathologica·Johannes BrettschneiderJohn Q Trojanowski
Nov 1, 2011·Annual Review of Pathology·Fadia IbrahimZissimos Mourelatos
Sep 26, 2012·Cellular and Molecular Life Sciences : CMLS·Petranka Krumova, Jochen H Weishaupt
Oct 22, 2010·Current Opinion in Neurology·Patricia Lillo, John R Hodges
Apr 24, 2012·Expert Opinion on Biological Therapy·Robert D DaytonRonald L Klein
Dec 4, 2010·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Yu-Ichi NotoSatoshi Kuwabara
Jun 23, 2015·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Koichi OkamotoMasamitsu Takatama
Nov 28, 2012·Physical Medicine and Rehabilitation Clinics of North America·Anuradha Duleep, Jeremy Shefner
May 2, 2015·Journal of Neuropathology and Experimental Neurology·Takahiro TakedaShinichiro Uchiyama
Nov 26, 2015·Neuroscience and Biobehavioral Reviews·Alida Spalloni, Patrizia Longone
Jan 23, 2013·Neuropathology and Applied Neurobiology·G C DeLucaG C Ebers
Dec 14, 2011·Brain Pathology·Christine Lund KraghEliezer Masliah

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