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 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.
Kostic
V
V
Department of Neurology, Columbia University, 650 West 168 Street, BB-307, New York, NY 10032, USA.
Jackson-Lewis
V
V
de Bilbao
F
F
Dubois-Dauphin
M
M
Przedborski
S
S
D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D000818:Animals; D004195:Disease Models, Animal; D005260:Female; D015870:Gene Expression; D019254:Genes, bcl-2; D015316:Genetic Therapy; D006801:Humans; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000473:pathology; D009410:Nerve Degeneration; D000090063:Proto-Oncogene Mas; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000502:physiology*; D016755:Proto-Oncogene Proteins c-jun / Q000032:analysis; D013116:Spinal Cord / Q000473:pathology; D013482:Superoxide Dismutase / Q000235:genetics / Q000378:metabolism; D015996:Survival Rate; D014452:Ubiquitins / Q000032:analysis
C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D019253:Proto-Oncogene Proteins c-bcl-2; D016755:Proto-Oncogene Proteins c-jun; D014452:Ubiquitins; D013482:Superoxide Dismutase