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Science 25 July 1997:
Vol. 277. no. 5325, pp. 559 - 563
DOI: 10.1126/science.277.5325.559
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Reports

Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

Vladimir Kostic, Vernice Jackson-Lewis, Fabienne de Bilbao, Michel Dubois-Dauphin, Serge Przedborski *

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-oncogene bcl-2 delayed 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 of bcl-2 also attenuated the magnitude of spinal cord motor neuron degeneration in the FALS-transgenic mice.

V. Kostic, V. Jackson-Lewis, S. Przedborski, Department of Neurology, Columbia University, 650 West 168 Street, BB-307, New York, NY 10032, USA.
F. de Bilbao and M. Dubois-Dauphin, Hôpitaux Universitaires de Genève, Division of Neuropsychiatry, 2 Chemin du Petit Bel Air, 1225 Geneva, Switzerland.
*   To whom correspondence should be addressed.

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