Publication Date:
1997-07-25
Description:
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.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Kostic, V -- Jackson-Lewis, V -- de Bilbao, F -- Dubois-Dauphin, M -- Przedborski, S -- NS01724/NS/NINDS NIH HHS/ -- New York, N.Y. -- Science. 1997 Jul 25;277(5325):559-62.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Neurology, Columbia University, 650 West 168 Street, BB-307, New York, NY 10032, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/9228005" target="_blank"〉PubMed〈/a〉
Keywords:
Amyotrophic Lateral Sclerosis/genetics/mortality/pathology/*therapy
;
Animals
;
Disease Models, Animal
;
Female
;
*Gene Expression
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*Genes, bcl-2
;
*Genetic Therapy
;
Humans
;
Male
;
Mice
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Mice, Transgenic
;
Motor Neurons/pathology
;
Nerve Degeneration
;
Proto-Oncogene Proteins c-bcl-2/*physiology
;
Proto-Oncogene Proteins c-jun/analysis
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Spinal Cord/pathology
;
Superoxide Dismutase/genetics/metabolism
;
Survival Rate
;
Ubiquitins/analysis
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
