FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 331:1721-1722 December 22, 1994 Number 25 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text Purchase this article Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited Related Article by Brown, R. H. PubMed Citation

To the Editor: The article by Dr. Brown (Oct. 20 issue)¹ highlights the studies that my colleagues and I have performed with a transgenic model of human motor neuron disease.² Our experiments built on the studies of Dr. Brown and other members of a multicenter group that identified mutations of copper-zinc superoxide dismutase in some patients with a dominantly inherited form of amyotrophic lateral sclerosis.³ In mice expressing mutant forms of human copper-zinc superoxide dismutase a progressive paralytic disease develops that causes death at four to five months of age. Symptoms are accompanied by vacuolar changes, mitochondrial cytopathologic changes, alterations . . . [Full Text of this Article]

References

This article has been cited by other articles:

• Prudencio, M., Hart, P. J., Borchelt, D. R., Andersen, P. M. (2009). Variation in aggregation propensities among ALS-associated variants of SOD1: Correlation to human disease. Hum Mol Genet 18: 3217-3226 [Abstract] [Full Text]  
• Wang, H., Ghosh, A., Baigude, H., Yang, C.-s., Qiu, L., Xia, X., Zhou, H., Rana, T. M., Xu, Z. (2008). Therapeutic Gene Silencing Delivered by a Chemically Modified Small Interfering RNA against Mutant SOD1 Slows Amyotrophic Lateral Sclerosis Progression. J. Biol. Chem. 283: 15845-15852 [Abstract] [Full Text]  
• Chang, Y., Stockinger, M. P., Tashiro, H., Lin, C.-l. G. (2008). A novel noncoding RNA rescues mutant SOD1-mediated cell death. FASEB J. 22: 691-702 [Abstract] [Full Text]  
• Fanara, P., Banerjee, J., Hueck, R. V., Harper, M. R., Awada, M., Turner, H., Husted, K. H., Brandt, R., Hellerstein, M. K. (2007). Stabilization of Hyperdynamic Microtubules Is Neuroprotective in Amyotrophic Lateral Sclerosis. J. Biol. Chem. 282: 23465-23472 [Abstract] [Full Text]  
• Gal, J., Strom, A.-L., Kilty, R., Zhang, F., Zhu, H. (2007). p62 Accumulates and Enhances Aggregate Formation in Model Systems of Familial Amyotrophic Lateral Sclerosis. J. Biol. Chem. 282: 11068-11077 [Abstract] [Full Text]  
• Vo, T. D., Palsson, B. O. (2007). Building the power house: recent advances in mitochondrial studies through proteomics and systems biology. Am. J. Physiol. Cell Physiol. 292: C164-C177 [Abstract] [Full Text]  
• Tummala, H., Jung, C., Tiwari, A., Higgins, C. M. J., Hayward, L. J., Xu, Z. (2005). Inhibition of Chaperone Activity Is a Shared Property of Several Cu,Zn-Superoxide Dismutase Mutants That Cause Amyotrophic Lateral Sclerosis. J. Biol. Chem. 280: 17725-17731 [Abstract] [Full Text]  
• Kirkinezos, I. G., Bacman, S. R., Hernandez, D., Oca-Cossio, J., Arias, L. J., Perez-Pinzon, M. A., Bradley, W. G., Moraes, C. T. (2005). Cytochrome c Association with the Inner Mitochondrial Membrane Is Impaired in the CNS of G93A-SOD1 Mice. J. Neurosci. 25: 164-172 [Abstract] [Full Text]  
• Broom, W. J., Parton, M. J., Vance, C. A., Russ, C., Andersen, P. M., Hansen, V., Leigh, P. N., Powell, J. F., Al-Chalabi, A., Shaw, C. E. (2004). No association of the SOD1 locus and disease susceptibility or phenotype in sporadic ALS. Neurology 63: 2419-2422 [Abstract] [Full Text]  
• Fukada, K., Zhang, F., Vien, A., Cashman, N. R., Zhu, H. (2004). Mitochondrial Proteomic Analysis of a Cell Line Model of Familial Amyotrophic Lateral Sclerosis. Mol. Cell. Proteomics 3: 1211-1223 [Abstract] [Full Text]  
• Andersen, J. (2003). Defects in Dynein Linked to Motor Neuron Degeneration in Mice. Sci Aging Knowl Environ 2003: pe10-10 [Abstract] [Full Text]  
• Bergeron, L., Perez, G. I., Macdonald, G., Shi, L., Sun, Y., Jurisicova, A., Varmuza, S., Latham, K. E., Flaws, J. A., Salter, J. C.M., Hara, H., Moskowitz, M. A., Li, E., Greenberg, A., Tilly, J. L., Yuan, J. (1998). Defects in regulation of apoptosis in caspase-2-deficient mice. Genes Dev. 12: 1304-1314 [Abstract] [Full Text]  
• Shinder, G. A., Lacourse, M.-C., Minotti, S., Durham, H. D. (2001). Mutant Cu/Zn-Superoxide Dismutase Proteins Have Altered Solubility and Interact with Heat Shock/Stress Proteins in Models of Amyotrophic Lateral Sclerosis. J. Biol. Chem. 276: 12791-12796 [Abstract] [Full Text]