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

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 tra...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 1997-07, Vol.277 (5325), p.559-562
Main Authors: Kostic, Vladimir, Jackson-Lewis, Vernice, de Bilbao, Fabienne, Dubois-Dauphin, Michel, Przedborski, Serge
Format: Article
Language:English
Subjects:
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - genetics
Amyotrophic Lateral Sclerosis - mortality
Amyotrophic Lateral Sclerosis - pathology
Amyotrophic Lateral Sclerosis - therapy
Anatomy
Animals
Axons
Biological and medical sciences
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Disease Models, Animal
End plates
Female
Gene Expression
Genes, bcl-2
Genetic mutation
Genetic Therapy
Humans
Male
Medical disorders
Medical research
Medical sciences
Mice
Mice, Transgenic
Motor neuron disease
Motor neurons
Motor Neurons - pathology
Nerve Degeneration
Neurology
Neuromuscular diseases
Neurons
Paralysis
Patients
Phrenic nerve
Proto-Oncogene Proteins c-bcl-2 - physiology
Proto-Oncogene Proteins c-jun - analysis
Rodents
Spinal cord
Spinal Cord - pathology
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Survival Rate
Transgenic animals
Ubiquitins - analysis
Ventilation
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Description
Summary: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.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.277.5325.559