| 18590823 |
Tsai LK, Tsai MS, Ting CH, Wang SH, Li H: Restoring Bcl-x (L) levels benefits a mouse model of spinal muscular atrophy. Neurobiol Dis. 2008 Sep;31(3):361-7. Epub 2008 Jun 3.
Currently, no curative treatment is available for spinal muscular atrophy (SMA). Since the degeneration of spinal motor neurons in SMA is mediated by apoptosis, over-expression of an anti-apoptotic factor, Bcl-x (L), may benefit SMA. Here, we crossed a mouse model of SMA with Bcl-x (L) transgenic mice to create SMA/Bcl-x (L) mice. The Bcl-x (L) expression in the spinal neurons of SMA/Bcl-x (L) mice was nearly double that in SMA mice. SMA/Bcl-x (L) mice showed preserved motor function, normalized electrophysiological tests, diminished muscle atrophy, and less motor neuron degeneration. In addition, the life span of SMA/Bcl-x (L) mice was 1.5 times longer than that of SMA mice. Therefore, over-expression of Bcl-x (L) has a potential for amelioration of SMA, and Bcl-x (L) may be another attractive therapeutic target other than survival motor neuron (SMN) protein for use in future drug screening for SMA. |
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