RT Journal Article SR Electronic T1 Bcl-2 overexpression prevents motoneuron cell body loss but not axonal degeneration in a mouse model of a neurodegenerative disease JF The Journal of Neuroscience JO J. Neurosci. FD Society for Neuroscience SP 7727 OP 7733 DO 10.1523/JNEUROSCI.15-11-07727.1995 VO 15 IS 11 A1 Sagot, Y A1 Dubois-Dauphin, M A1 Tan, SA A1 de Bilbao, F A1 Aebischer, P A1 Martinou, JC A1 Kato, AC YR 1995 UL http://www.jneurosci.org/content/15/11/7727.abstract AB 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.