Lost in translation: treatment trials in the SOD1 mouse and in human ALS - PubMed (original) (raw)
Review
Lost in translation: treatment trials in the SOD1 mouse and in human ALS
Michael Benatar. Neurobiol Dis. 2007 Apr.
Abstract
Therapeutic success in the superoxide dismutase (SOD1) mouse model of amyotrophic lateral sclerosis (ALS) has not translated into effective therapy for human ALS, calling into question the utility of such preclinical data for identifying therapeutic agents that are worthy of further study in humans. This random effects meta-analysis of treatment trials in the superoxide dismutase (SOD1) mouse was undertaken in order to explore possible reasons for this failure of translational research and to identify potential pharmacological interventions that might be used in either a preventative or therapeutic trial in familial ALS. Among studies in which treatment was initiated presymptomatically, the weighted mean differences (WMDs) comparing the active treatment to control treated animals were 12 days (onset), 13 days (survival) and 5 days (survival interval). Among studies in which treatment was initiated at the time of symptom onset, the WMDs were 15 days (survival) and 8 days (survival interval). Subgroup analysis suggests that drugs such as minocycline and Cox-2 inhibitors with an anti-inflammatory mechanism of action, and anti-oxidative agents such as creatine or the manganese porphyrin AEOL-10150, appear to be the most promising for preventative and therapeutic trials respectively in patients with familial ALS. These conclusions should be tempered by the methodological limitations of the relevant literature.
Similar articles
- Therapeutic immunization with a glatiramer acetate derivative does not alter survival in G93A and G37R SOD1 mouse models of familial ALS.
Haenggeli C, Julien JP, Mosley RL, Perez N, Dhar A, Gendelman HE, Rothstein JD. Haenggeli C, et al. Neurobiol Dis. 2007 Apr;26(1):146-52. doi: 10.1016/j.nbd.2006.12.013. Epub 2006 Dec 30. Neurobiol Dis. 2007. PMID: 17276077 - Motor neuron dysfunction in a mouse model of ALS: gender-dependent effect of P2X7 antagonism.
Cervetto C, Frattaroli D, Maura G, Marcoli M. Cervetto C, et al. Toxicology. 2013 Sep 6;311(1-2):69-77. doi: 10.1016/j.tox.2013.04.004. Epub 2013 Apr 11. Toxicology. 2013. PMID: 23583883 - Vascular endothelial growth factor prolongs survival in a transgenic mouse model of ALS.
Zheng C, Nennesmo I, Fadeel B, Henter JI. Zheng C, et al. Ann Neurol. 2004 Oct;56(4):564-7. doi: 10.1002/ana.20223. Ann Neurol. 2004. PMID: 15389897 - Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS.
Turner BJ, Talbot K. Turner BJ, et al. Prog Neurobiol. 2008 May;85(1):94-134. doi: 10.1016/j.pneurobio.2008.01.001. Epub 2008 Jan 16. Prog Neurobiol. 2008. PMID: 18282652 Review. - Pharmacological treatment of ALS.
Münch C, Ludolph AC. Münch C, et al. Neurol Neurochir Pol. 2001;35(1 Suppl):41-50. Neurol Neurochir Pol. 2001. PMID: 11732279 Review.
Cited by
- Dexpramipexole is ineffective in two models of ALS related neurodegeneration.
Vieira FG, LaDow E, Moreno A, Kidd JD, Levine B, Thompson K, Gill A, Finkbeiner S, Perrin S. Vieira FG, et al. PLoS One. 2014 Dec 19;9(12):e91608. doi: 10.1371/journal.pone.0091608. eCollection 2014. PLoS One. 2014. PMID: 25526593 Free PMC article. - How clinical trials of myasthenia gravis can inform pre-clinical drug development.
Rostedt Punga A, Kaminski HJ, Richman DP, Benatar M. Rostedt Punga A, et al. Exp Neurol. 2015 Aug;270:78-81. doi: 10.1016/j.expneurol.2014.12.022. Epub 2015 Jan 13. Exp Neurol. 2015. PMID: 25592627 Free PMC article. - Aggregated SOD1 causes selective death of cultured human motor neurons.
Benkler C, O'Neil AL, Slepian S, Qian F, Weinreb PH, Rubin LL. Benkler C, et al. Sci Rep. 2018 Nov 6;8(1):16393. doi: 10.1038/s41598-018-34759-z. Sci Rep. 2018. PMID: 30401824 Free PMC article. - Current and emerging treatments for amyotrophic lateral sclerosis.
Zoccolella S, Santamato A, Lamberti P. Zoccolella S, et al. Neuropsychiatr Dis Treat. 2009;5:577-95. doi: 10.2147/ndt.s7788. Epub 2009 Nov 16. Neuropsychiatr Dis Treat. 2009. PMID: 19966906 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Research Materials
Miscellaneous