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Science 6 November 1998:
Vol. 282. no. 5391, pp. 1079 - 1083
DOI: 10.1126/science.282.5391.1079
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Review

Genetic Neurodegenerative Diseases: The Human Illness and Transgenic Models

Donald L. Price, * Sangram S. Sisodia, David R. Borchelt

The neurodegenerative disorders, a heterogeneous group of chronic progressive diseases, are among the most puzzling and devastating illnesses in medicine. Some of these disorders, such as Alzheimer's disease, amyotrophic lateral sclerosis, the prion diseases, and Parkinson's disease, can occur sporadically and, in some instances, are caused by inheritance of gene mutations. Huntington's disease is acquired in an entirely genetic manner. Transgenic mice that express disease-causing genes recapitulate many features of these diseases. This review provides an overview of transgenic mouse models of familial amyotrophic lateral sclerosis, familial Alzheimer's disease, and Huntington's disease and the emerging insights relevant to the underlying molecular mechanisms of these diseases.

D. L. Price is in the Departments of Pathology, Neurology, and Neuroscience and the Neuropathology Laboratory, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. S. S. Sisodia is in the Department of Pharmacological and Physiological Sciences, University of Chicago, Chicago, IL 60637, USA. D. R. Borchelt is in the Department of Pathology and the Neuropathology Laboratory, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
*   To whom correspondence should be addressed at the Division of Neuropathology, Johns Hopkins University School of Medicine, 558 Ross Research Building, 720 Rutland Avenue, Baltimore, Maryland 21205-2196, USA. E-mail: dlprice{at}welchlink.welch.jhu.edu

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