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M. Leitner, S. Menzies and C. Lutz, Working with ALS Mice: Guidelines for preclinical testing & colony management.

Summary:  This manual summarize the current best practices and recommendations available for (1) designing and conducting preclinical studies using the currently available SOD1-based mouse models of ALS and (2) breeding and maintaining SOD1 mutant mouse colonies. The manual was prepared through a joint collaboration with The Jackson Laboratory and Prize4Life, Inc.


Boillee, S., C. Vande Velde, and D.W. Cleveland, ALS: A Disease of Motor Neurons and their Non-Neuronal Neighbors. Neuron, 2006. 52: pp 39-59.

Summary: This review highlights the involvement of nonneuronal cells in ALS disease progression and particularly the role of non-cell-autonomous processes in motor neuron killing. Concludes with a discussion of potential therapeutic approaches aimed at nonneuronal cells.


Bruijn, L.I., T.M. Miller, and D.W. Cleveland, Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci, 2004. 27: p. 723-49.

Summary: This early review discusses the various known ALS-related mutations (circa 2004) and the development of model systems, based on these mutations, for testing hypotheses of disease mechanism and potential therapies.


Clark, J., Pritchard, C., and Sunak, S. on behalf of ALSTDF. Amyotrophic Lateral Sclerosis: A report on the state of research into the cause, cure, and prevention of ALS. Report to the MA Dept of Health, 2005. pp 1-359.

Summary: This is a very detailed and well-written meta-review of ALS epidemiology, clinical information, and research topics (circa 2005). Because it is divided into sections, it is possible to focus in on select topics of interest (rather than read the entire lengthy document).


Gwinn K, Corriveau RA, Mitsumoto H, Bednarz K, Brown RH, et al (2007) Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery. PLoS ONE 2(12): e1254. doi:10.1371/journal.pone.0001254

Summary: This article discusses a large collaboration among academic investigators, government, and non-government organizations which has created a public repository of human DNA, immortalized cell lines, and clinical data to further gene discovery in ALS. This resource currently maintains samples and associated phenotypic data from 2332 MND subjects and 4692 controls.


Pasinelli, P., and Brown, R.H., Molecular Biology of Amyotrophic Lateral Sclerosis: Insights from Genetics. Nat Neurosci Rev, 2006. 7: pp 710-723.

Summary: This review focuses on the genetics of ALS and extrapolates from the growing number of ALS-associated genes to the diverse molecular pathways involved in ALS pathogenesis.


Traynor, B.J., et al. Neuroprotective agents for clinical trials in ALS: A systematic assessment. Neurology, 2006. 67: pp 20-27.

Summary: This review identifies potential neuroprotective agents (circa 2006), representing a wide range of mechanisms, for testing in phase III clinical trials and outlines which data still need to be collected for each drug.