Design, power, and interpretation of studies in the standard murine model of ALS
Scott, Sean, Kranz, Janice E., Cole, Jeff, Lincecum, John M., Thompson, Kenneth, Kelly, Nancy, Bostrom, Alan, Theodoss, Jill +4 more·2008·Amyotrophic Lateral Sclerosis
Cited by (503)
Items 201–300 of 503
- CuATSM efficacy is independently replicated in a SOD1 mouse model of ALS while unmetallated ATSM therapy fails to reveal benefitsFernando G. Vieira, Theo Hatzipetros, Kenneth Thompson, Andy J. Moreno, Joshua D. Kidd, Valerie R. Tassinari, Beth A. Levine, Steven Perrin, Alan Gill · 2017 · IBRO Reports
- Effect of Sex on Lifespan, Disease Progression, and the Response to Methionine Sulfoximine in the SOD1 G93A Mouse Model for ALSMonica Bame, Patricia A. Pentiak, Richard B. Needleman, William S. A. Brusilow · 2012 · Gender Medicine
- Use of biomarkers in ALS drug development and clinical trialsNadine Bakkar, Ashley V. Boehringer, Robert P. Bowser · 2014 · Brain Research
- Death receptor 6 (DR6) antagonist antibody is neuroprotective in the mouse SOD1G93A model of amyotrophic lateral sclerosisGuanrong Huang, X Lee, Yuting Bian, Zhaohui Shao, G.-N. Sheng, R. Blake Pepinsky, Sha Mi · 2013 · Cell Death and Disease
- Therapeutic Strategies Under Development Targeting Inflammatory Mechanisms in Amyotrophic Lateral SclerosisSebastiano Giuseppe Crisafulli, Simona Brajkovic, Maria Sara Cipolat Mis, Valeria Parente, Stefania Paola Corti · 2017 · Molecular Neurobiology
- Redox Modifier Genes and Pathways in Amyotrophic Lateral SclerosisBarrie J. Carter, Pervin N. Anklesaria, Stephanie H. Choi, John F. Engelhardt · 2009 · Antioxidants and Redox Signaling
- The molecular tweezer CLR01 inhibits aberrant superoxide dismutase 1 (SOD1) self-assembly in vitro and in the G93A-SOD1 mouse model of ALSRavinder Malik, Helen Meng, Piriya Wongkongkathep, Christian I. Corrales, Niki Sepanj, Ryan Saleh Atlasi, Frank‐Gerrit Klärner, Thomas H. Schrader, Melissa J. Spencer, Joseph A. Loo et al. · 2019 · Journal of Biological Chemistry
- Adenylyl Cyclase type 3, a marker of primary cilia, is reduced in primary cell culture and in lumbar spinal cord in situ in G93A SOD1 miceXiaoxing Ma, Randy Peterson, John Turnbull · 2011 · BMC Neuroscience
- A brief review of recent Charcot-Marie-Tooth research and prioritiesSean Ekins, Nadia K. Litterman, Renée J.G. Arnold, Robert W. Burgess, Joel S. Freundlich, Steven James Gray, Joseph J Higgins, Brett C. Langley, Dianna E. Willis, Lucia M. Notterpek et al. · 2015 · F1000Research
- Non-Invasive MRI and Spectroscopy of mdx Mice Reveal Temporal Changes in Dystrophic Muscle Imaging and in Energy DeficitsChristopher R. Heier, Alfredo Daniel Guerron, Alexandru V. Korotcov, Stephen Lin, Heather Gordish‐Dressman, Stanley Thomas Fricke, Raymond W. Sze, Eric P. Hoffman, Paul C. Wang, Kanneboyina Nagaraju · 2014 · PLoS ONE
- Reduced VGLUT2 expression increases motor neuron viability in Sod1G93A miceHanna Wootz, Anders Enjin, Åsa Wallén‐Mackenzie, Dan Lindholm, Klas Kullander · 2009 · Neurobiology of Disease
- A natural human IgM that binds to gangliosides is therapeutic in murine models of amyotrophic lateral sclerosisXiaohua Xu, Aleksandar Đenić, Luke R. Jordan, Nathan J. Wittenberg, Arthur E. Warrington, Bharath Wootla, Louisa M. Papke, Laurie J. Zoecklein, Daehan Yoo, Jonah Shaver et al. · 2015 · Disease Models & Mechanisms
- Out of Control? Managing Baseline Variability in Experimental Studies with Control GroupsPaul C. Moser · 2019 · Handbook of experimental pharmacology
- Expression profile of pattern recognition receptors in skeletal muscle of SOD1 (G93A) amyotrophic lateral sclerosis (ALS) mice and sporadic ALS patientsSwen Lehmann, E. Esch, P. Hartmann, Anand Goswami, Stefan Nikolin, Joachim Weis, Cordian Beyer, Sonja Johann · 2018 · Neuropathology and Applied Neurobiology
- Light-Activated Dynamic Clamp Using iPSC-Derived CardiomyocytesBonnie Quach, Trine Krogh‐Madsen, Emilia G. Entcheva, David J. Christini · 2018 · Biophysical Journal
- Determining sample size adequacy for animal model studies in nutrition research: limits and ethical challenges of ordinary power calculation proceduresC. Ricci, J. Baumgartner, L. Malan, C. Smuts · 2020 · International Journal of Food Science and Nutrition
- Therapy development for ALS: Lessons learned and path forwardVeena Lanka, Merit Cudkowicz · 2008 · Amyotrophic Lateral Sclerosis
- Inappropriate modeling of chronic and complex disorders: How to reconsider the approach in the context of predictive, preventive and personalized medicine, and translational medicineSoroush Seifirad, Vahid Haghpanah · 2019 · The EPMA Journal
- The P2X7 receptor antagonist JNJ-47965567 administered thrice weekly from disease onset does not alter progression of amyotrophic lateral sclerosis in SOD1G93A miceDiane Ly, Anjila Dongol, Peter Cuthbertson, Thomas V. Guy, Nicholas J. Geraghty, Reece Andrew Sophocleous, Lucia Sin, Bradley J. Turner, Debbie Watson, Justin J. Yerbury et al. · 2020 · Purinergic Signalling
- Relevance of transgenic mouse models for Alzheimer's diseaseLeon M. Tai, Juan Maldonado Weng, Mary Jo LaDu, Scott T. Brady · 2020 · Progress in molecular biology and translational science
- Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP‐43 pathologyBarış Genç, Mukesh Gautam, Öge Gözütok, Ina Dervishi, Santana S. Sanchez, Gashaw M. Goshu, Nuran Koçak, Edward F. Xie, Richard B. Silverman, P. Hande Özdi̇nler · 2021 · Clinical and Translational Medicine
- Riluzole does not ameliorate disease caused by cytoplasmic TDP‐43 in a mouse model of amyotrophic lateral sclerosisAmanda L. Wright, Paul A. Della Gatta, Sheng Le, Britt A. Berning, Prachi M. Mehta, Kelly R. Jacobs, Hossai Gul, Rebecca San Gil, Thomas J. Hedl, Winonah R. Riddell et al. · 2021 · European Journal of Neuroscience
- Human Pluripotent Stem Cells: Applications and Challenges in Neurological DiseasesYoussef Hibaoui, Anis Féki · 2012 · Frontiers in Physiology
- Unraveling the Complexity of Amyotrophic Lateral Sclerosis: Recent Advances from the Transgenic Mutant SOD1 MiceMarco Peviani, Ilaria Caron, Chiara Pizzasegola, Francesco Gensano, Massimo Tortarolo, Caterina Bendotti · 2010 · CNS & Neurological Disorders - Drug Targets
- Questions regarding the predictive value of one evolved complex adaptive system for a second: Exemplified by the SOD1 mouseRay Greek, Lawrence A. Hansen · 2013 · Progress in Biophysics and Molecular Biology
- Microglia RAGE exacerbates the progression of neurodegeneration within the SOD1G93A murine model of amyotrophic lateral sclerosis in a sex-dependent mannerMacLean, Michael, Juranek, Judyta, Cuddapah, Swetha, López-Díez, Raquel, Ruiz, Henry H., Hu, Jiyuan, Frye, Laura, Li, Huilin, Gugger, Paul F., Schmidt, Ann Marie · 2021 · Journal of Neuroinflammation
- Modeling Amyotrophic Lateral Sclerosis in hSOD1G93A Transgenic SwineMaria Novella Chieppa, Andrea Perota, Cristiano Corona, Anna Grindatto, Irina Lagutina, Elena Vallino Costassa, Giovanna Lazzari, S. Colleoni, Roberto Duchi, Franco Lucchini et al. · 2013 · Neurodegenerative Diseases
- Androgen receptor antagonism accelerates disease onset in the SOD1 G93A mouse model of amyotrophic lateral sclerosisVictoria M. McLeod, Chew Ling Lau, Mathew D. F. Chiam, Thusitha Rupasinghe, Ute Roessner, Elvan Djouma, Wah Chin Boon, Bradley J. Turner · 2019 · British Journal of Pharmacology
- The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALSYulia Dzhashiashvili, Chase P. Monckton, Harini Shah, Rejani B. Kunjamma, Brian Popko · 2019 · Neurobiology of Disease
- Targeting mitochondrial dysfunction in amyotrophic lateral sclerosis: a systematic review and meta-analysisArpan R. Mehta, Rachel Walters, Fergal M. Waldron, Suvankar Pal, Bhuvaneish Thangaraj Selvaraj, Malcolm Robert Macleod, Giles E. Hardingham, Siddharthan Chandran, Jenna M. Gregory · 2019 · Brain Communications
- Mitochondria: A Promising Convergent Target for the Treatment of Amyotrophic Lateral SclerosisTeresa Cunha‐Oliveira, Liliana Montezinho, Rui F. Simões, Marcelo de Carvalho, Elisabete B. Ferreiro, Filomena S. G. Silva · 2024 · Cells
- Moving forward in clinical trials for ALS: motor neurons lead the way pleaseBarış Genç, P. Hande Özdi̇nler · 2013 · Drug Discovery Today
- Transplantation of mesenchymal stem cells in ALSLetizia Mazzini, Alessandro Vercelli, Ivana Ferrero, Marina Boido, Roberto M. Cantello, Franca Fagioli · 2012 · Progress in brain research
- Association of ROP18 and ROP5 was efficient as a marker of virulence in atypical isolates of Toxoplasma gondii obtained from pigs and goats in Piauí, BrazilWagner Martins Fontes do Rêgo, Jean-Marc Costa, Ramon Castro de Araújo Baraviera, Lorena Velozo Pinto, Gabriella de Lima Bessa, Rosálida Estevam Nazar Lopes, Ricardo Wagner Almeida Vítor · 2017 · Veterinary Parasitology
- Modelling amyotrophic lateral sclerosis in miceJodie Stephenson, Sandra Amor · 2017 · Drug Discovery Today Disease Models
- Copper-ATSM as a Treatment for ALS: Support from Mutant SOD1 Models and BeyondSara Nikseresht, James B. W. Hilton, Kai Kysenius, Jeffrey R. Liddell, Peter John Crouch · 2020 · Life
- Does the preclinical evidence for functional remyelination following myelinating cell engraftment into the injured spinal cord support progression to clinical trials?Scott A. Myers, Andrew N. Bankston, Darlene A. Burke, Sujata Saraswat Ohri, Scott R. Whittemore · 2016 · Experimental Neurology
- Why Are So Many Epidemiology Associations Inflated or Wrong? Does Poorly Conducted Animal Research Suggest Implausible Hypotheses?Michael B. Bracken · 2009 · Annals of Epidemiology
- Dietary Supplementation with S-Adenosyl Methionine Delays the Onset of Motor Neuron Pathology in a Murine Model of Amyotrophic Lateral SclerosisJames J. Suchy, Sangmook Lee, Ambar Ahmed, Thomas B. Shea · 2009 · NeuroMolecular Medicine
- Status of ALS treatment, insights into therapeutic challenges and dilemmasKhamaysa, Mohammed, Pradat, Pierre-François · 2022 · Journal of Personalized Medicine
- Identifying and understanding factors that affect the translation of therapies from the laboratory to patients: a study protocolManoj Mathew Lalu, Joshua Ryan Montroy, C. Glenn Begley, Tania M. Bubela, Victoria T. Hunniford, David Ripsman, Neil L. Wesch, Jonathan Kimmelman, Malcolm Robert Macleod, David Moher et al. · 2020 · F1000Research
- Glutamatergic Neuroplasticity in Cocaine AddictionJoachim D. K. Uys, Kathryn J. Reissner · 2011 · Progress in molecular biology and translational science
- Regulation of Intracellular Copper by Induction of Endogenous Metallothioneins Improves the Disease Course in a Mouse Model of Amyotrophic Lateral SclerosisEiichi Tokuda, Shunsuke Watanabe, Eriko Okawa, Shin-ichi Ono · 2015 · Neurotherapeutics
- Control of neuroinflammation as a therapeutic strategy for amyotrophic lateral sclerosis and other neurodegenerative disordersRodney Lee Mosley, Howard Eliot Gendelman · 2010 · Experimental Neurology
- Preclinical efficacy in therapeutic area guidelines from the U.S. Food and Drug Administration and the European Medicines Agency: a cross‐sectional studyHolger Langhof, W. W. Chin, S. Wieschowski, Carole A. Federico, J. Kimmelman, D. Strech · 2018 · British Journal of Pharmacology
- TRICALS: creating a highway toward a cureRuben P. A. van Eijk, Tessa Kliest, Christopher J. McDermott, Kit C. B. Roes, Philip Van Damme, Adriano Chiò, Markus Weber, Caroline Ingre, Philippe Corcia, Mónica Povedano et al. · 2020 · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
- Adipose-derived stem cells protect motor neurons and reduce glial activation in both in vitro and in vivo models of ALSYuri Ciervo, Noemi Gatto, Chloe F. Allen, Andrew J. Grierson, Laura Ferraiuolo, Richard J. Mead, Pamela J. Shaw · 2021 · Molecular Therapy — Methods & Clinical Development
- How significant are your data? The need for a culture shiftMartin Christian Michel · 2014 · Naunyn-Schmiedeberg s Archives of Pharmacology
- Pathophysiology, Clinical Heterogeneity, and Therapeutic Advances in Amyotrophic Lateral Sclerosis: A Comprehensive Review of Molecular Mechanisms, Diagnostic Challenges, and Multidisciplinary Management StrategiesMaría González-Sánchez, Marı́a Jesús Ramı́rez-Expósito, José Manuel Martı́nez-Martos · 2025 · Life
- Preclinical Evidence for the Efficacy of Ischemic Postconditioning against Renal Ischemia-Reperfusion Injury, a Systematic Review and Meta-AnalysisSimone J. Jonker, Theo P. Menting, Michiel C. Warlé, Merel J. Ritskes-Hoitinga, Kimberley Elaine Wever · 2016 · PLoS ONE
- Therapeutic blockade of HMGB1 reduces early motor deficits, but not survival in the SOD1G93A mouse model of amyotrophic lateral sclerosisJohn D. Lee, Ning Liu, Samantha C. Levin, Lars Ottosson, Ulf Andersson, Helena Erlandsson Harris, Trent M. Woodruff · 2019 · Journal of Neuroinflammation
- The methodological quality of animal research in critical care: the public face of scienceMeredith Bara, Ari Robin Joffe · 2014 · Annals of Intensive Care
- Lack of synergistic effect of resveratrol and sigma-1 receptor agonist (PRE-084) in SOD1G93A ALS mice: overlapping effects or limited therapeutic opportunity?Renzo Mancuso, Jaume del Valle, Marta Morell, Mercè Pallàs, Rosario Osta, Xavier Navarro · 2014 · Orphanet Journal of Rare Diseases
- Focus on the Role of D-serine and D-amino Acid Oxidase in Amyotrophic Lateral Sclerosis/Motor Neuron Disease (ALS)Nazanin Rahmani Kondori, Praveen Paul, Jacqueline P. Robbins, Ke Liu, John C. W. Hildyard, Dominic J. Wells, Jacqueline de Belleroche · 2018 · Frontiers in Molecular Biosciences
- Improving translatability of preclinical studies for neuromuscular disorders: lessons from the TREAT-NMD Advisory Committee for Therapeutics (TACT)Raffaella Willmann, Joanne Lee, Cathy Turner, Kanneboyina Nagaraju, Annemieke M Aartsma-Rus, Dominic J. Wells, Kathryn R. Wagner, Cristina Csimma, Volker W. Straub, Miranda D. Grounds et al. · 2020 · Disease Models & Mechanisms
- Mouse Models of Neurodegenerative Diseases: Criteria and General MethodologyChristopher Janus, Hans Welzl · 2009 · Methods in molecular biology
- Functional improvement in mouse models of familial amyotrophic lateral sclerosis by PEGylated insulin-like growth factor I treatment depends on disease severityStefanie Saenger, Bettina Holtmann, Mark R. Nilges, Susanne Schroeder, Andreas Hoeflich, Heidemarie Kletzl, Will P. J. M. Spooren, Susanne Ostrowitzki, Taleen Hanania, Michael Sendtner et al. · 2012 · Amyotrophic Lateral Sclerosis
- Yeast genetic screen reveals novel therapeutic strategy for ALSMatthew D. Figley, Aaron D. Gitler · 2013 · Rare Diseases
- Axonal and neuromuscular synaptic phenotypes in Wld, SOD1 and ostes mutant mice identified by fiber-optic confocal microendoscopyFrances Wong, Li Fan, Sara E. Wells, Robert Hartley, Francesca E. Mackenzie, Oyinlola O Oyebode, Rosalind Brown, Derek Thomson, Michael P. Coleman, Gonzalo Blanco et al. · 2009 · Molecular and Cellular Neuroscience
- Amyotrophic Lateral Sclerosis: Drug Therapy from the Bench to the BedsideMark B. Bromberg, Summer B. Gibson · 2012 · Seminars in Neurology
- Epothilone D accelerates disease progression in the SOD1G93A mouse model of amyotrophic lateral sclerosisJayden A. Clark, Catherine A. Blizzard, Monique C. Breslin, Elise J. Yeaman, K. M. Lee, Jyoti A. Chuckowree, Tracey C. Dickson · 2018 · Neuropathology and Applied Neurobiology
- Review: Microglia in motor neuron diseaseBridget Ashford, Delphine Boche, Johnathan Cooper‐Knock, Paul Roy Heath, Julie E. Simpson, J. Robin Highley · 2020 · Neuropathology and Applied Neurobiology
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- Food restriction-induced autophagy modulates degradation of mutant SOD1 in an amyotrophic lateral sclerosis mouse modelKunxi Zhang, Pengxiao Shi, Ting An, Qian Wang, Jingjing Wang, Zhongyao Li, Weisong Duan, Chunyan Li, Yansu Guo · 2013 · Brain Research
- Intraspinal bone-marrow cell therapy at pre- and symptomatic phases in a mouse model of amyotrophic lateral sclerosisFernanda Gubert, Ana B. Decotelli, Igor Bonacossa-Pereira, Fernanda R. Figueiredo, Camila Zaverucha-do-Valle, Fernanda Freire Tovar-Moll, Luísa Hoffmann, Turán Peter Urmenyi, Marcelo F. Santiago, Rosalia Méndez‐Otero · 2016 · Stem Cell Research & Therapy
- Gene therapy for amyotrophic lateral sclerosisThais Federici, Nicholas M. Boulis · 2011 · Neurobiology of Disease
- Large-scale drug screening in iPSC-derived motor neurons from sporadic ALS patients identifies a potential combinatorial therapyChristopher R. Bye, Elizabeth Ling Qian, Katherine Lim, Maciej Daniszewski, Fleur C. Garton, Bảo C. Trần-Lê, Helena Hai Liang, Tian Lin, John George Lock, Duncan E. Crombie et al. · 2025 · Nature Neuroscience
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- The Risk-Escalation Model: A Principled Design Strategy for Early-Phase TrialsSpencer Phillips Hey, Jonathan Kimmelman · 2014 · Kennedy Institute of Ethics journal
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