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ALS Clinical Trials Review: 20 Years of Failure. Are We Any Closer to Registering a New Treatment?
Open accessAbstract
Amyotrophic lateral sclerosis (ALS) is a devastating condition with an estimated mortality of 30,000 patients a year worldwide. The median reported survival time since onset ranges from 24 to 48 months. Riluzole is the only currently approved mildly efficacious treatment. Riluzole received marketing authorization in 1995 in the USA and in 1996 in Europe. In the years that followed, over 60 molecules have been investigated as a possible treatment for ALS. Despite significant research efforts, the overwhelming majority of human clinical trials (CTs) have failed to demonstrate clinical efficacy. In the past year, oral masitinib and intravenous edaravone have emerged as promising new therapeutics with claimed efficacy in CTs in ALS patients. Given their advanced phase of clinical development one may consider these drugs as the most likely near-term additions to the therapeutic arsenal available for patients with ALS. In terms of patient inclusion, CT with masitinib recruited a wider, more representative, less restrictive patient population in comparison to the only successful edaravone CT (edaravone eligibility criteria represents only 18% of masitinib study patients). The present manuscript reviews >50 CTs conducted in the last 20 years since riluzole was first approved. A special emphasis is put on the analysis of existing evidence in support of the clinical efficacy of edaravone and masitinib and the possible implications of an eventual marketing authorisation in the treatment of ALS.
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“Multiple small molecules with anti-inflammatory activity, including minocycline, masitinib, NP001, and celecoxib, have been tested for the treatment of ALS. Although most have failed, masitinib was found to be effective in the preceding clinical trials, and a Phase III clinical trial testing masitinib in ALS patients is ongoing.577”
From Role of neuroinflammation in neurodegeneration development (2023) · cites this paper as [577]
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“Probiotics are considered to be dietary factors that can influence the human intestinal microbiota, which can affect the composition and structure of the intestinal flora; which, in turn, plays a role in protecting the intestinal epithelial barrier, inhibiting pathogen production, and regulating the immune system (3).”
From Modulation of the gut–brain axis via the gut microbiota: a new era in treatment of amyotrophic lateral sclerosis (2023) · cites this paper as [3]
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“Through use of probiotics, the number of SCFA-producing microorganisms in the intestine can be increased (3).”
From Modulation of the gut–brain axis via the gut microbiota: a new era in treatment of amyotrophic lateral sclerosis (2023) · cites this paper as [3]
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Cites
Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation
Gurney, M. E., Pu, H., Chiu, A. Y., Dal Canto, M. C., Polchow, C. Y., Alexander, D. D., Caliendo, J., Hentati, A., Kwon, Y. W., Deng, H.-X., et al. (1994) · Science · doi:10.1126/science.8209258 · Cited by 4,143
Kiernan, M. C., Vucic, S., Cheah, B. C., Turner, M. R., Eisen, A., Hardiman, O., Burrell, J. R., & Zoing, M. C. (2011) · The Lancet · doi:10.1016/s0140-6736(10)61156-7 · Cited by 2,603
A Controlled Trial of Riluzole in Amyotrophic Lateral Sclerosis
Bensimon, G., Lacomblez, L., & Meininger, V. (1994) · New England Journal of Medicine · doi:10.1056/nejm199403033300901 · Cited by 2,239
β-Lactam antibiotics offer neuroprotection by increasing glutamate transporter expression
Rothstein, J. D., Patel, S., Regan, M. R., Haenggeli, C., Huang, Y. H., Bergles, D. E., Jin, L., Dykes Hoberg, M., Vidensky, S., Chung, D. S., et al. (2005) · Nature · doi:10.1038/nature03180 · Cited by 1,518
Dose-ranging study of riluzole in amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis/Riluzole Study Group II, Lacomblez, L., Bensimon, G., Meininger, V., Leigh, P. N., & Guillet, P. (1996) · The Lancet · doi:10.1016/s0140-6736(96)91680-3 · Cited by 1,303
Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)
Miller, R. G., Mitchell, J. D., & Moore, D. H. (2012) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd001447.pub3 · Cited by 1,123
Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)
Miller, R. G., Jd, M., Lyon, M., & Moore, D. (2002) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd001447 · Cited by 910
Oxidative Stress in Neurodegenerative Diseases
Niedzielska, E., Smaga, I., Gawlik, M., Moniczewski, A., Stankowicz, P., Pera, J., & Filip, M. (2016) · Molecular Neurobiology · doi:10.1007/s12035-015-9337-5 · Cited by 800
Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
Klivenyi, P., Ferrante, R. J., Matthews, R. T., Bogdanov, M. B., Klein, A. M., Andreassen, O. A., Mueller, G., Wermer, M., Kaddurah-Daouk, R., & Beal, M. F. (1999) · Nature Medicine · doi:10.1038/6568 · Cited by 682
Preclinical research: Make mouse studies work
Perrin, S. (2014) · Nature · doi:10.1038/507423a · Cited by 668
Research works printed but not linked: 5
- ARISLA (Fondazione Italiana de Ricerca Per La Sclerosi Laterale), 2014, ALS Clinical Trials List.
- Bradley, 1995, A phase I/II study of recombinant brain-derived neurotrophic in patients with ALS., Ann. Neurol., 38
- Rosenfeld, 2001, “Creatine monohydrate in amyotrophic lateral sclerosis: preliminary results,” in Proceedings of the, American Academy of Neurology Annual Meeting
- Teva, 2010, News Release.
- Tohgi, 1999, Remarkable increase in cerebrospinal fluid 3-nitrotyrosine in patients with sporadic amyotrophic lateral sclerosis., Ann. Neurol., 46, 129, 10.1002/1531-8249(199907)46:1129::AID-ANA213.0.CO;2-Y · DOI
Cited by
Sorted by citations
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Hou, Y., Dan, X., Babbar, M., Wei, Y., Hasselbalch, S. G., Croteau, D. L., & Bohr, V. A. (2019) · Nature Reviews Neurology · doi:10.1038/s41582-019-0244-7 · Cited by 3,310
Role of neuroinflammation in neurodegeneration development
Zhang, W., Xiao, D., Mao, Q., & Xia, H. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01486-5 · Cited by 1,668
“Although most have failed, masitinib was found to be effective in the preceding clinical trials, and a Phase III clinical trial testing masitinib in ALS patients is ongoing.(577) dl-3-nButylphthalide (dl-NBP), a synthetic compound based on l-3-nButylphthalide that is isolated from seeds of Apium graveolens, has been f…”
BackgroundMolecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K. (2019) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2019.00025 · Cited by 810
ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?
Mejzini, R., Flynn, L. L., Pitout, I. L., Fletcher, S., Wilton, S. D., & Akkari, P. A. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.01310 · Cited by 803
Mead, R. J., Shan, N., Reiser, H. J., Marshall, F., & Shaw, P. J. (2023) · Nature Reviews Drug Discovery · doi:10.1038/s41573-022-00612-2 · Cited by 505
CNS inflammation and neurodegeneration
Chitnis, T., & Weiner, H. L. (2017) · Journal of Clinical Investigation · doi:10.1172/jci90609 · Cited by 470
Liu, J., & Wang, F. (2017) · Frontiers in Immunology · doi:10.3389/fimmu.2017.01005 · Cited by 376
Amyotrophic Lateral Sclerosis: An Update for 2018
Oskarsson, B., Gendron, T. F., & Staff, N. P. (2018) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2018.04.007 · Cited by 338
Genetics of amyotrophic lateral sclerosis: A review
Mathis, S., Goizet, C., Soulages, A., Vallat, J., & Masson, G. L. (2019) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2019.02.030 · Cited by 267
Neurovascular Unit Dysfunction and Neurodegenerative Disorders
Yu, X., Ji, C., & Shao, A. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.00334 · Cited by 244
Mora, J. S., Genge, A. L., Chiò, A., Estol, C. J., Chaverri, D., Hernández, M., Marín, S., Cadavid, J. M., Rodriguez, G. E., Povedano, M., et al. (2019) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2019.1632346 · Cited by 233
Self-citationRNA Dysregulation in Amyotrophic Lateral Sclerosis
Butti, Z., & Patten, S. A. (2019) · Frontiers in Genetics · doi:10.3389/fgene.2018.00712 · Cited by 208
Gene therapy for ALS: A review
Amado, D. A., & Davidson, B. L. (2021) · Molecular Therapy · doi:10.1016/j.ymthe.2021.04.008 · Cited by 203
Current State and Future Directions in the Therapy of ALS
Tzeplaeff, L., Wilfling, S., Requardt, M. V., & Herdick, M. L. (2023) · Cells · doi:10.3390/cells12111523 · Cited by 168
Molecular and Cellular Mechanisms Affected in ALS
Gall, L. L., Anakor, E., Connolly, O., Vijayakumar, U. G., Duddy, W. J., & Duguez, S. (2020) · Journal of Personalized Medicine · doi:10.3390/jpm10030101 · Cited by 152
Oeckl, P., Weydt, P., Steinacker, P., Anderl-Straub, S., Nordin, F., Volk, A. E., Diehl-Schmid, J., Andersen, P. M., Kornhuber, J., Danek, A., et al. (2019) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2018-318868 · Cited by 139
Small-molecule dissolution of stress granules by redox modulation benefits ALS models
Uechi, H., Sridharan, S., Nijssen, J., Bilstein, J., Iglesias‐Artola, J. M., Kishigami, S., Casablancas-Antràs, V., Poser, I., Martinez, E. J., Boczek, E. E., et al. (2025) · Nature Chemical Biology · doi:10.1038/s41589-025-01893-5 · Cited by 139
Obrador, E., Palmer, R. S., López‐Blanch, R., Jihad-Jebbar, A., Vallés, S. L., & Estrela, J. M. (2020) · Antioxidants · doi:10.3390/antiox9090901 · Cited by 132
Jin, M., Günther, R., Akgün, K., Hermann, A., & Ziemssen, T. (2020) · Scientific Reports · doi:10.1038/s41598-020-62756-8 · Cited by 130
Béland, L.-C., Markovinovic, A., Jakovac, H., De Marchi, F., Bilic, E., Mazzini, L., Kriz, J., & Munitic, I. (2020) · Brain Communications · doi:10.1093/braincomms/fcaa124 · Cited by 125
Neurotheranostics as Personalized Medicines
Kevadiya, B. D., Ottemann, B. M., Thomas, M. B., Mukadam, I., Nigam, S., McMillan, J., Gorantla, S., Bronich, T. K., Edagwa, B., & Gendelman, H. E. (2019) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2018.10.011 · Cited by 124
Neuromuscular Junction Dysfunction in Amyotrophic Lateral Sclerosis
Verma, S., Khurana, S., Vats, A., Sahu, B., Ganguly, N. K., Chakraborti, P., Gourie-Devi, M., & Taneja, V. (2022) · Molecular Neurobiology · doi:10.1007/s12035-021-02658-6 · Cited by 126
Oeckl, P., Weydt, P., Thal, D. R., Weishaupt, J. H., Ludolph, A. C., & Otto, M. (2019) · Acta Neuropathologica · doi:10.1007/s00401-019-02093-x · Cited by 120
Wiesenfarth, M., Dorst, J., Brenner, D., Elmas, Z. G., Parlak, Ö., Uzelac, Ž., Kandler, K., Mayer, K., Weiland, U., Herrmann, C. H., et al. (2024) · EClinicalMedicine · doi:10.1016/j.eclinm.2024.102495 · Cited by 122
New Insights into Oxidative Stress and Inflammatory Response in Neurodegenerative Diseases
Scarian, E., Viola, C., Dragoni, F., Gerlando, R. D., Rizzo, B., Diamanti, L., Gagliardi, S., Bordoni, M., & Pansarasa, O. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25052698 · Cited by 118
Disease-modifying therapies in amyotrophic lateral sclerosis
Chiò, A., Mazzini, L., & Mora, G. (2020) · Neuropharmacology · doi:10.1016/j.neuropharm.2020.107986 · Cited by 122
Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis
McGurk, L., Mojsilovic‐Petrovic, J., Deerlin, V. M. V., Shorter, J., Kalb, R. G., Lee, V. M., Trojanowski, J. Q., Lee, E. B., & Bonini, N. M. (2018) · Acta Neuropathologica Communications · doi:10.1186/s40478-018-0586-1 · Cited by 113
Clinical Trials in Traumatic Spinal Cord Injury
Donovan, J. M., & Kirshblum, S. (2018) · Neurotherapeutics · doi:10.1007/s13311-018-0632-5 · Cited by 110
Aymerich, M. S., Aso, E., Abellanas, M. A., Tolón, R. M., Ramos, J. A., Ferrer, I., Romero, J., & Fernández‐Ruíz, J. (2018) · Biochemical Pharmacology · doi:10.1016/j.bcp.2018.08.016 · Cited by 113
Risk factors and emerging therapies in amyotrophic lateral sclerosis
Nowicka, N., Juranek, J., Juranek, J. K., & Wojtkiewicz, J. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20112616 · Cited by 106
Calzaferri, F., Ruiz‐Ruiz, C., de Diego, A. M. G., de Pascual, R., Méndez‐López, I., Cano‐Abad, M. F., Maneu, V., Ríos, C. D. L., Gandía, L., & de Diego, A. M. G. (2020) · Medicinal Research Reviews · doi:10.1002/med.21710 · Cited by 101
Correlating serum micrornas and clinical parameters in amyotrophic lateral sclerosis
Raheja, R., Regev, K., Healy, B. C., Mazzola, M. A., Beynon, V., von Glehn, F., Paul, A., Diaz‐Cruz, C., Gholipour, T., Glanz, B. I., et al. (2018) · Muscle & Nerve · doi:10.1002/mus.26106 · Cited by 100
Gene Therapy for ALS—A Perspective
Cappella, M., Ciotti, C., Cohen-Tannoudji, M., & Biferi, M. G. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20184388 · Cited by 103
A patent review of histone deacetylase 6 inhibitors in neurodegenerative diseases (2014-2019)
Shen, S., & Kozikowski, A. P. (2019) · Expert Opinion on Therapeutic Patents · doi:10.1080/13543776.2019.1708901 · Cited by 96
The Peripheral Immune System and Amyotrophic Lateral Sclerosis
McCombe, P., Lee, J. D., Woodruff, T. M., & Henderson, R. D. (2020) · Frontiers in Neurology · doi:10.3389/fneur.2020.00279 · Cited by 100
Chipika, R. H., Finegan, E., Shing, S. L. H., Hardiman, O. M., & Bede, P. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00229 · Cited by 93
Mitochondrial Dysfunctions: A Red Thread across Neurodegenerative Diseases
Stanga, S., Caretto, A., Boido, M., & Vercelli, A. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21103719 · Cited by 90
A Pivotal Role of Nrf2 in Neurodegenerative Disorders: A New Way for Therapeutic Strategies
Süzen, S., Tucci, P., Profumo, E., Buttari, B., & Saso, L. (2022) · Pharmaceuticals · doi:10.3390/ph15060692 · Cited by 90
Targeting the nucleolus as a therapeutic strategy in human disease
Corman, A., Sirozh, O., Lafarga, V., & Fernández-Capetillo, Ó. (2022) · Trends in Biochemical Sciences · doi:10.1016/j.tibs.2022.09.006 · Cited by 89
Antioxidant Alternatives in the Treatment of Amyotrophic Lateral Sclerosis: A Comprehensive Review
Carrera‐Juliá, S., Moreno, M. L., Barrios, C., Ortí, J. E. D. L. R., & Drehmer, E. (2020) · Frontiers in Physiology · doi:10.3389/fphys.2020.00063 · Cited by 87
Overexpression of ferroptosis defense enzyme Gpx4 retards motor neuron disease of SOD1G93A mice
Chen, L., Na, R., McLane, K. D., Thompson, C. S., Gao, J., Wang, X., & Ran, Q. (2021) · Scientific Reports · doi:10.1038/s41598-021-92369-8 · Cited by 84
Bono, S., Feligioni, M., & Corbo, M. (2021) · Molecular Neurodegeneration · doi:10.1186/s13024-021-00479-8 · Cited by 85
Human pluripotent stem cell–derived models and drug screening in CNS precision medicine
Silva, M. C., & Haggarty, S. J. (2019) · Annals of the New York Academy of Sciences · doi:10.1111/nyas.14012 · Cited by 87
Advances in nanotechnology-based strategies for the treatments of amyotrophic lateral sclerosis
Wang, G., Rayner, S. L., Chung, R. S., Shi, B., & Liang, X. J. (2020) · Materials Today Bio · doi:10.1016/j.mtbio.2020.100055 · Cited by 84
Swindell, W. R., Kruse, C. P. S., List, E. O., Berryman, D. E., & Kopchick, J. J. (2019) · Journal of Translational Medicine · doi:10.1186/s12967-019-1909-0 · Cited by 80
Vu, L. T., An, J., Kovalik, T., Gendron, T. F., Petrucelli, L., & Bowser, R. P. (2020) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2019-321916 · Cited by 85
Magi, S., Piccirillo, S., & Amoroso, S. (2019) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-018-3002-x · Cited by 79
Izrael, M., Slutsky, S. G., Admoni, T., Cohen, L., Granit, A., Hasson, A., Itskovitz‐Eldor, J., Paker, L. K., Kuperstein, G., Lavon, N., et al. (2018) · Stem Cell Research & Therapy · doi:10.1186/s13287-018-0890-5 · Cited by 74
Dash, R. P., Babu, R. J., & Srinivas, N. R. (2018) · Clinical Pharmacokinetics · doi:10.1007/s40262-018-0655-4 · Cited by 73
Masrori, P., Beckers, J., Gossye, H., & Damme, P. V. (2022) · Molecular Neurodegeneration · doi:10.1186/s13024-022-00525-z · Cited by 80
Human Pluripotent Stem Cells in Neurodegenerative Diseases: Potentials, Advances and Limitations
Kolagar, T. A., Farzaneh, M., Nikkar, N., & Khoshnam, S. E. (2019) · Current Stem Cell Research & Therapy · doi:10.2174/1574888x14666190823142911 · Cited by 71
Fernández‐Ruíz, J. (2018) · British Journal of Pharmacology · doi:10.1111/bph.14382 · Cited by 70
Protein aggregation and therapeutic strategies in SOD1- and TDP-43- linked ALS
Tsekrekou, M., Giannakou, M., Papanikolopoulou, K., & Σκρέτας, Γ. (2024) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2024.1383453 · Cited by 71
Implications of Microglia in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Haukedal, H., & Freude, K. (2019) · Journal of Molecular Biology · doi:10.1016/j.jmb.2019.02.004 · Cited by 68
Synaptic restoration by cAMP/PKA drives activity-dependent neuroprotection to motoneurons in ALS
Bączyk, M., Alami, N. O., Delestrée, N., Martinot, C., Tang, L., Commisso, B., Bayer, D., Doisne, N., Frankel, W. N., Manuel, M., et al. (2020) · The Journal of Experimental Medicine · doi:10.1084/jem.20191734 · Cited by 70
Exciting Complexity: The Role of Motor Circuit Elements in ALS Pathophysiology
Gunes, Z. I., Kan, V. W. Y., Ye, X., & Liebscher, S. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.00573 · Cited by 74
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Zhao, J., He, Z., & Wang, J. (2021) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2021.771898 · Cited by 66
Decoding hand movements from human EEG to control a robotic arm in a simulation environment
Schwarz, A., Höller, M., Pereira, J., Ofner, P., & Müller-Putz, G. (2020) · Journal of Neural Engineering · doi:10.1088/1741-2552/ab882e · Cited by 51
ALS longitudinal studies with frequent data collection at home: study design and baseline data
Rutkove, S. B., Qi, K., Shelton, K., Liss, J., Berisha, V., & Shefner, J. M. (2018) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2018.1541095 · Cited by 66
Voltage-Gated Sodium Channel Dysfunctions in Neurological Disorders
Barbieri, R., Nizzari, M., Zanardi, I., Pusch, M., & Gavazzo, P. (2023) · Life · doi:10.3390/life13051191 · Cited by 65
Harnessing neuroplasticity: modern approaches and clinical future
Sasmita, A. O., Kuruvilla, J., & Ling, A. P. K. (2018) · International Journal of Neuroscience · doi:10.1080/00207454.2018.1466781 · Cited by 69
Rodríguez‐Cueto, C., Santos‐García, I., García‐Toscano, L., Espejo‐Porras, F., Bellido, M., Fernández‐Ruíz, J., Muñóz, E., & de Lago, E. (2018) · Biochemical Pharmacology · doi:10.1016/j.bcp.2018.07.049 · Cited by 66
Sigma 1 receptor as a therapeutic target for amyotrophic lateral sclerosis
Herrando‐Grabulosa, M., Gaja‐Capdevila, N., Vela, J. M., & Navarro, X. (2020) · British Journal of Pharmacology · doi:10.1111/bph.15224 · Cited by 64
Mitochondria, a Key Target in Amyotrophic Lateral Sclerosis Pathogenesis
Genin, E. C., Abou-Ali, M., & Paquis‐Flucklinger, V. (2023) · Genes · doi:10.3390/genes14111981 · Cited by 64
Petrou, P., Kassis, I., Yaghmour, N. E., Ginzberg, A., & Karussis, D. (2021) · Frontiers in Bioscience-Landmark · doi:10.52586/4980 · Cited by 61
Neuroimmune and Inflammatory Signals in Complex Disorders of the Central Nervous System
Liberman, A. C., Trías, E., Chagas, L. D. S., Trindade, P., Pereira, M. D. S., Refojo, D., Hedin‐Pereira, C., & Serfaty, C. A. (2018) · NeuroImmunoModulation · doi:10.1159/000494761 · Cited by 62
Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells
Guo, W., Fumagalli, L., Prior, R. C., & Bosch, L. V. D. (2017) · Frontiers in Neuroscience · doi:10.3389/fnins.2017.00671 · Cited by 65
Riluzole does not improve lifespan or motor function in three ALS mouse models
Hogg, M. C., Halang, L., Woods, I., Coughlan, K. S., & Prehn, J. H. M. (2017) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2017.1407796 · Cited by 61
Orr, B. O., Hauswirth, A. G., Celona, B., Fetter, R. D., Zunino, G., Kvon, E. Z., Zhu, Y., Pennacchio, L. A., Black, B. L., & Davis, G. W. (2020) · Neuron · doi:10.1016/j.neuron.2020.04.009 · Cited by 62
Clinically Precedented Protein Kinases: Rationale for Their Use in Neurodegenerative Disease
Benn, C., & Dawson, L. A. (2020) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2020.00242 · Cited by 59
Cognata, V. L., Morello, G., & Cavallaro, S. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22094820 · Cited by 57
Aziz, A., Noreen, S., Khalid, W., Mubarik, F., Niazi, M. K., Koraqi, H., Ali, A., Lima, C. M. G., Alansari, W. S., Eskandrani, A. A., et al. (2022) · Molecules · doi:10.3390/molecules27217320 · Cited by 56
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Ediriweera, G. R., Chen, L., Yerbury, J. J., Thurecht, K. J., & Vine, K. L. (2021) · Molecular Pharmaceutics · doi:10.1021/acs.molpharmaceut.1c00297 · Cited by 57
Reduced retinal nerve fiber layer (RNFL) thickness in ALS patients: a window to disease progression
Rohani, M., Meysamie, A., Zamani, B., Sowlat, M., & Akhoundi, F. H. (2018) · Journal of Neurology · doi:10.1007/s00415-018-8863-2 · Cited by 55
Axonal regeneration and sprouting as a potential therapeutic target for nervous system disorders
Marshall, K. L., & Farah, M. H. (2021) · Neural Regeneration Research · doi:10.4103/1673-5374.308077 · Cited by 59
Islam, F., Islam, M. R., Meem, A. F. K., Nafady, M. H., Islam, M. R., Akter, A., Mitra, S., Alhumaydhi, F. A., Emran, T. B., Khusro, A., et al. (2022) · Chemosphere · doi:10.1016/j.chemosphere.2022.136020 · Cited by 55
RetractedWang, Y., Liu, F.-T., Wang, Y., Guan, R., Chen, C., Li, D.-K., Bu, L.-L., Song, J., Yang, Y., Dong, Y., et al. (2018) · Neurotoxicity Research · doi:10.1007/s12640-018-9865-7 · Cited by 53
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Garcia‐Gancedo, L., Kelly, M. L., Lavrov, A., Parr, J., Hart, R. G., Marsden, R., Turner, M. R., Talbot, K., Chiwera, T., Shaw, C. E., et al. (2019) · JMIR mhealth and uhealth · doi:10.2196/13433 · Cited by 53
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Li, T., Martin, E., Abada, Y.-S. K., Boucher, C., Cès, A., Youssef, I., Fenaux, G., Forand, Y., Legrand, A., Nadkarni, N. A., et al. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-200466 · Cited by 52
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Zubiri, I., Lombardi, V., Bremang, M., Mitra, V., Nardo, G., Adiutori, R., Lu, C., Leoni, E., Yip, P. K., Yildiz, Ö., et al. (2018) · Molecular Neurodegeneration · doi:10.1186/s13024-018-0292-2 · Cited by 43
Metadata
- Authors
- Petrov, D., Mansfield, C., Moussy, A., & Hermine, O.
- Affiliations
- AB Science (France) (FR)
- Hôpital Necker-Enfants Malades (FR)
- Corresponding author
- Hermine, Olivier
- Venue
- Frontiers in Aging Neuroscience
- Publisher
- Frontiers Media SA
- Published
- 2017-03-22
- Volume
- 9
- Pages
- 68-68
- ISSN
- 1663-4365 (ISSN-L)
- PMC
- PMC5360725
- OpenAlex
- W2597399186
- Cited by
- 451
- References
- 108
- Citations by year
- 2026: 28 · 2025: 41 · 2024: 45 · 2023: 46 · 2022: 65 · 2021: 63 · 2020: 53 · 2019: 52 · 2018: 50 · 2017: 8
- FWCI
- 5.94
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by
- Locations
- Frontiers in Aging Neuroscience (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Funders
- Agence Nationale de la Recherche (ANR-11-LABX-0051)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Genetics (clinical) › Neurogenetic and Muscular Disorders Research
- Keywords
- amyotrophic lateral sclerosis, clinical trial failure, riluzole, edaravone, masitinib, drug development, clinical efficacy, patient selection criteria, drug approval, neurodegenerative disease therapeutics
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