Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis
10.1126/science.277.5325.559
Crossref journal-article
American Association for the Advancement of Science (AAAS)
Science (221)
Abstract

Mutations in the gene encoding copper/zinc superoxide dismutase enzyme produce an animal model of familial amyotrophic lateral sclerosis (FALS), a fatal disorder characterized by paralysis. Overexpression of the proto-oncogenebcl-2delayed onset of motor neuron disease and prolonged survival in transgenic mice expressing the FALS-linked mutation in which glycine is substituted by alanine at position 93. It did not, however, alter the duration of the disease. Overexpression ofbcl-2also attenuated the magnitude of spinal cord motor neuron degeneration in the FALS-transgenic mice.

Bibliography

Kostic, V., Jackson-Lewis, V., de Bilbao, F., Dubois-Dauphin, M., & Przedborski, S. (1997). Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis. Science, 277(5325), 559–563.

Authors 5
  1. Vladimir Kostic (first)
  2. Vernice Jackson-Lewis (additional)
  3. Fabienne de Bilbao (additional)
  4. Michel Dubois-Dauphin (additional)
  5. Serge Przedborski (additional)
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  51. We thank A. B. Naini and R. Yokoyama for performing the SOD assays M. Schoenebeck for preparing phrenic nerve sections M. Gurney for kindly providing the information about the number of copies in the transgenic G93A mice and the G1H mice and R. E. Burke and C. M. Troy for their insightful comments on the manuscript. This work was supported by the Muscular Dystrophy Association the National Institute of Neurological Disorders and Stroke (grant NS01724) the Swiss National Foundation (grant 31-4331695) the Lowenstein Foundation and the Parkinson's Disease Foundation. S.P. is a recipient of the Irving A. Hansen Memorial Foundation Award and the Cotzias Award from the American Parkinson Disease Association.
Dates
Type When
Created 23 years, 1 month ago (July 27, 2002, 5:44 a.m.)
Deposited 8 months, 3 weeks ago (Dec. 8, 2024, 1:34 p.m.)
Indexed 1 month, 1 week ago (July 25, 2025, 6:24 a.m.)
Issued 28 years, 1 month ago (July 25, 1997)
Published 28 years, 1 month ago (July 25, 1997)
Published Print 28 years, 1 month ago (July 25, 1997)
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@article{Kostic_1997, title={Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis}, volume={277}, ISSN={1095-9203}, url={http://dx.doi.org/10.1126/science.277.5325.559}, DOI={10.1126/science.277.5325.559}, number={5325}, journal={Science}, publisher={American Association for the Advancement of Science (AAAS)}, author={Kostic, Vladimir and Jackson-Lewis, Vernice and de Bilbao, Fabienne and Dubois-Dauphin, Michel and Przedborski, Serge}, year={1997}, month=jul, pages={559–563} }