Genetically engineered mouse models of neurodegenerative diseases

ABSTRACT Recent research has significantly advanced our understanding of the molecular mechanisms of neurodegenerative diseases, including Alzheimer's disease (AD) and motor neuron

disease. Here we emphasize the use of genetically engineered mouse models that are instrumental for understanding why AD is a neuronal disease, and for validating attractive therapeutic

targets. In motor neuron diseases, Cu/Zn superoxide dismutase and survival motor neuron mouse models are useful in testing disease mechanisms and therapeutic strategies for amyotrophic

lateral sclerosis (ALS) and spinal motor atrophy, respectively, but the mechanisms that account for selective motor neuron loss remain uncertain. We anticipate that, in the future, therapies

based on understanding disease mechanisms will be identified and tested in mouse model systems. Access through your institution Buy or subscribe This is a preview of subscription content,

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The authors thank colleagues from JHMI, particularly S. Sisodia, M. Lee, G. Thinakaren, E. Koo, J. Subramaniam, L. Martin, V. Koliatsos, A. Bergin, L. Brujin, C. Pardo, B. Rabin, T.

Crawford, M. Becher, P. Hoffman, J. Griffin, J. Rothstein, J. Troncoso, T. Li, V. Culotta and D. Cleveland as well as those at other institutions (J. Gitlin) for contributions to the

original work cited in this review and for discussions. Supported by grants from the U. S. Public Health Service (AG05146, AG07914, AG10480, AG10491, AG14248, NS07435, NS20471, NS37145,

NS10580, NS37771, NS40014, NS38377, NS38065) as well as the Metropolitan Life Foundation, Adler Foundation, and Bristol-Myers Squibb Foundation. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS *

Department of Pathology, The Johns Hopkins University School of Medicine, 558 Ross Research Building, 720 Rutland Avenue, Baltimore, 21205-2196, Maryland, USA Philip C. Wong, Huaibin Cai, 

David R. Borchelt & Donald L. Price * Department of Neuroscience, The Johns Hopkins University School of Medicine, 558 Ross Research Building, 720 Rutland Avenue, Baltimore, 21205-2196,

Maryland, USA Philip C. Wong, Huaibin Cai, David R. Borchelt & Donald L. Price * Department of Neurology, The Johns Hopkins University School of Medicine, 558 Ross Research Building, 720

Rutland Avenue, Baltimore, 21205-2196, Maryland, USA Donald L. Price * The Division of Neuropathology, The Johns Hopkins University School of Medicine, 558 Ross Research Building, 720

Rutland Avenue, Baltimore, 21205-2196, Maryland, USA Philip C. Wong, Huaibin Cai, David R. Borchelt & Donald L. Price Authors * Philip C. Wong View author publications You can also

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models of neurodegenerative diseases. _Nat Neurosci_ 5, 633–639 (2002). https://doi.org/10.1038/nn0702-633 Download citation * Received: 14 January 2002 * Accepted: 30 May 2002 * Issue Date:

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