Most cited papers in Amyotrophic Lateral Sclerosis Research
What are the most cited papers in Amyotrophic Lateral Sclerosis Research?
The most cited paper in Amyotrophic Lateral Sclerosis Research is "Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis" (Daniel R. Rosen et al., 1993), with 7,116 citations. Next come "Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis" (6,756) and "El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis" (5,487).
Showing 50 of 100 papers. Every paper with more than 1,057 citations is listed.
| # | Paper | ||
|---|---|---|---|
| 1 | Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis Daniel R. Rosen et al. · Nature | Mar 1993 | 7,116 |
| 2 | Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis Manuela Neumann, Deepak M. Sampathu, Linda K. Kwong et al. · Science | 5 Oct 2006 | 6,756 |
| 3 | El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis Benjamin Rix Brooks, Robert G. Miller, Michael Swash et al. · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders | 2000 | 5,487 |
| 4 | Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia Katya Rascovsky et al. · Brain | 2 Aug 2011 | 5,386 |
| 5 | Global prevalence of dementia: a Delphi consensus study Cleusa P. Ferri, Martin James Prince, Carol Brayne et al. · The Lancet | Dec 2005 | 5,347 |
| 6 | Frontotemporal lobar degeneration David Neary, Julie S. Snowden, Lars Gustafson et al. · Neurology | Dec 1998 | 5,100 |
| 7 | Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS Mariely DeJesus‐Hernandez et al. · Neuron | 21 Sep 2011 | 5,066 |
| 8 | A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD Alan Edward Renton et al. · Neuron | 22 Sep 2011 | 4,545 |
| 9 | Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation Mark E. Gurney, Haifeng Pu, Arlene Y. Chiu et al. · Science | 17 Jun 1994 | 4,179 |
| 10 | Neurodegenerative diseases and oxidative stress Kevin Jeffrey Barnham, Colin Louis Masters, Ashley Ian Bush · Nature Reviews Drug Discovery | Mar 2004 | 3,710 |
| 11 | The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function Jesse M. Cedarbaum, Nancy Stambler, Errol Malta et al. · Journal of the Neurological Sciences | Oct 1999 | 3,349 |
| 12 | A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation Avinash Patel, Hyun O. Lee, Louise M. Jawerth et al. · Cell | Aug 2015 | 3,277 |
| 13 | Second consensus statement on the diagnosis of multiple system atrophy Sid Gilman, Gregor Karl Wenning, Phillip A. Low et al. · Neurology | 25 Aug 2008 | 3,194 |
| 14 | TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Tetsuaki Arai, Masato Hasegawa, Haruhiko Akiyama et al. · Biochemical and Biophysical Research Communications | 31 Oct 2006 | 2,838 |
| 15 | TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis Jemeen Sreedharan, Ian P. Blair, Vineeta B. Tripathi et al. · Science | 28 Feb 2008 | 2,678 |
| 16 | Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6 Caroline A. Vance et al. · Science | 26 Feb 2009 | 2,624 |
| 17 | Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis Thomas J. Kwiatkowski et al. · Science | 26 Feb 2009 | 2,597 |
| 18 | Amyotrophic lateral sclerosis Matthew C. Kiernan, Steve Vucic, Benjamin C. Cheah et al. · The Lancet | 5 Feb 2011 | 2,529 |
| 19 | Single-Dose Gene-Replacement Therapy for Spinal Muscular Atrophy Jerry R. Mendell et al. · New England Journal of Medicine | Nov 2017 | 2,411 |
| 20 | Neurodegenerative Tauopathies Virginia M.‐Y. Lee, Michel Goedert, John Q. Trojanowski · Annual Review of Neuroscience | Mar 2001 | 2,407 |
| 21 | Nusinersen versus Sham Control in Infantile-Onset Spinal Muscular Atrophy Richard S. Finkel et al. · New England Journal of Medicine | Nov 2017 | 2,357 |
| 22 | A Controlled Trial of Riluzole in Amyotrophic Lateral Sclerosis Gilbert Bensimon, Lucette Lacomblez, Vincent Meininger · New England Journal of Medicine | 3 Mar 1994 | 2,231 |
| 23 | Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expression Igor N. Zelko, Thomas J. Mariani, Rodney J. Folz · Free Radical Biology and Medicine | Aug 2002 | 2,217 |
| 24 | Amyotrophic Lateral Sclerosis Robert H. Brown, Ammar Al‐Chalabi · New England Journal of Medicine | 12 Jul 2017 | 2,195 |
| 25 | Decoding ALS: from genes to mechanism J. Paul Taylor, Robert H. Brown, Don W. Cleveland · Nature | 8 Nov 2016 | 2,158 |
| 26 | Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17 Matt C. Baker et al. · Nature | 16 Jul 2006 | 2,131 |
| 27 | Neurofilaments as biomarkers in neurological disorders Michael Khalil, Charlotte Elisabeth Teunissen, Markus Otto et al. · Nature Reviews Neurology | 31 Aug 2018 | 2,042 |
| 28 | How neuroinflammation contributes to neurodegeneration Richard M. Ransohoff · Science | 18 Aug 2016 | 2,021 |
| 29 | Scale for the assessment and rating of ataxia T. Schmitz-Hübsch et al. · Neurology | 12 Jun 2006 | 1,999 |
| 30 | Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons John T. Dimos, Kit T. Rodolfa, Kathy K. Niakan et al. · Science | 31 Jul 2008 | 1,999 |
| 31 | Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis Shuo‐Chien Ling, Magdalini Polymenidou, Don W. Cleveland · Neuron | Aug 2013 | 1,887 |
| 32 | Amyotrophic Lateral Sclerosis Lewis P. Rowland, Neil A. Shneider · New England Journal of Medicine | 31 May 2001 | 1,840 |
| 33 | Hallmarks of neurodegenerative diseases David M. Wilson, Mark Cookson, Ludo Van Den Bosch et al. · Cell | Feb 2023 | 1,784 |
| 34 | Placebo-Controlled Phase 3 Study of Oral BG-12 for Relapsing Multiple Sclerosis Ralf Gold, Ludwig Kappos, Douglas Lorne Arnold et al. · New England Journal of Medicine | 19 Sep 2012 | 1,735 |
| 35 | Oligodendroglia metabolically support axons and contribute to neurodegeneration Youngjin Lee, Brett M. Morrison, Yun Li et al. · Nature | Jul 2012 | 1,693 |
| 36 | Undetectable Intracellular Free Copper: The Requirement of a Copper Chaperone for Superoxide Dismutase Tracey Rae, Paul Joseph Schmidt, Robert A. Pufahl et al. · Science | 30 Apr 1999 | 1,642 |
| 37 | PINK1-dependent recruitment of Parkin to mitochondria in mitophagy Cristòfol Vives-Bauzá, Chun Hui Zhou, Yong Huang et al. · Proceedings of the National Academy of Sciences | 6 Dec 2009 | 1,632 |
| 38 | Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia Séverine Boillée, Koji Yamanaka, Christian S. Lobsiger et al. · Science | Jun 2006 | 1,629 |
| 39 | Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report Peter T. Nelson et al. · Brain | 22 Mar 2019 | 1,619 |
| 40 | Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets Chao Gao, Jingwen Jiang, Yuyan Tan et al. · Signal Transduction and Targeted Therapy | 21 Sep 2023 | 1,581 |
| 41 | State of play in amyotrophic lateral sclerosis genetics Alan Edward Renton, Adriano Chiò, Bryan J. Traynor · Nature Neuroscience | 26 Dec 2013 | 1,576 |
| 42 | TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis Edor Kabashi, Paul N. Valdmanis, Patrick A. Dion et al. · Nature Genetics | 30 Mar 2008 | 1,559 |
| 43 | Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21 Marc Cruts et al. · Nature | 16 Jul 2006 | 1,546 |
| 44 | Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS Hong Joo Kim et al. · Nature | Mar 2013 | 1,535 |
| 45 | Clinical and Pathological Diagnosis of Frontotemporal Dementia Guy M. McKhann · Archives of Neurology | Nov 2001 | 1,493 |
| 46 | Amyotrophic Lateral Ssclerosis and Structural Defects in Cu,Zn Superoxide Dismutase Han‐Xiang Deng, Afif Hentati, John A. Tainer et al. · Science | 20 Aug 1993 | 1,483 |
| 47 | Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosis Jeffrey D. Rothstein, Marleen Van Kammen, Allan I. Levey et al. · Annals of Neurology | Jul 1995 | 1,483 |
| 48 | Nusinersen versus Sham Control in Later-Onset Spinal Muscular Atrophy Eugenio Maria Mercuri et al. · New England Journal of Medicine | 14 Feb 2018 | 1,466 |
| 49 | Expression of a noncoding RNA is elevated in Alzheimer's disease and drives rapid feed-forward regulation of β-secretase Mohammad Ali Faghihi, Farzaneh Modarresi, Ahmad Mohammad Khalil et al. · Nature Medicine | 29 Jun 2008 | 1,457 |
| 50 | Amyotrophic lateral sclerosis Orla M. Hardiman, Ammar Al‐Chalabi, Adriano Chiò et al. · Nature Reviews Disease Primers | 5 Oct 2017 | 1,451 |
Citations are OpenAlex's own counts, from its 2026-09-23 snapshot, so the ranking can differ from Google Scholar or Web of Science. A paper is listed for a field when OpenAlex files it under one of the field's topics; retracted papers and papers whose citation count cannot be traced to citing works are left out, and a preprint and the article it became are shown once. Release dates are OpenAlex's: where a source gave only a month or a year, that is what is shown. How the lists are chosen.