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-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.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Amyotrophic Lateral Sclerosis / genetics
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Amyotrophic Lateral Sclerosis / mortality
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Amyotrophic Lateral Sclerosis / pathology
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Amyotrophic Lateral Sclerosis / therapy*
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Animals
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Disease Models, Animal
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Female
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Gene Expression*
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Genes, bcl-2*
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Genetic Therapy*
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Humans
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Male
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Mice
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Mice, Transgenic
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Motor Neurons / pathology
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Nerve Degeneration
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Proto-Oncogene Mas
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Proto-Oncogene Proteins c-bcl-2 / physiology*
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Proto-Oncogene Proteins c-jun / analysis
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Spinal Cord / pathology
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Superoxide Dismutase / genetics
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Superoxide Dismutase / metabolism
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Survival Rate
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Ubiquitins / analysis
Substances
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MAS1 protein, human
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Proto-Oncogene Mas
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Proto-Oncogene Proteins c-bcl-2
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Proto-Oncogene Proteins c-jun
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Ubiquitins
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Superoxide Dismutase