Bcl-2 overexpression prevents motoneuron cell body loss but not axonal degeneration in a mouse model of a neurodegenerative disease
| Autor: | S. A. Tan, J.-C. Martinou, P. Aebischer, Y. Sagot, A. C. Kato, F. De Bilbao, Michel Dubois-Dauphin |
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| Rok vydání: | 1995 |
| Předmět: |
Genetically modified mouse
Cell Mice Transgenic Degeneration (medical) Biology Proto-Oncogene Mas Mice Pons Proto-Oncogene Proteins NMNAT1 medicine Animals Humans Motor Neuron Disease Motor Neurons Cell Death Oncogene General Neuroscience Articles Motor neuron Immunohistochemistry Choline acetyltransferase Axons Cell biology Phrenic Nerve Facial Nerve medicine.anatomical_structure Proto-Oncogene Proteins c-bcl-2 nervous system Apoptosis Nerve Degeneration Neuroscience |
| Zdroj: | The Journal of Neuroscience. 15:7727-7733 |
| ISSN: | 1529-2401 0270-6474 |
| DOI: | 10.1523/jneurosci.15-11-07727.1995 |
| Popis: | Bcl-2 and its analogs protect different classes of neurons from apoptosis in several experimental situations. These proteins may therefore provide a means for treatment of neurodegenerative diseases. We examined the effects of Bcl-2 overexpression in a genetic mouse model with motor neuron disease (progressive motor neuronopathy/pmn). Pmn/pmn mice lose motoneurons and myelinated axons, and die at 6 weeks of age. When these mice were crossed with transgenic mice that overexpress human Bcl-2, there was a rescue of the facial motoneurons with a concomitant restoration of their normal soma size and expression of choline acetyltransferase. However, Bcl-2 overexpression did not prevent degeneration of myelinated axons in the facial and phrenic motor nerves and it did not increase the life span of the animals. Since Bcl-2 acts strictly on neuronal cell body survival without compensating for nerve degeneration in pmn/pmn/bcl-2 mice, this proto- oncogene would not in itself be sufficient for treatment of neurodegenerative diseases where axonal impairment is a major component. |
| Databáze: | OpenAIRE |
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