| First Author | Tsai MS | Year | 2006 |
| Journal | Eur J Hum Genet | Volume | 14 |
| Issue | 3 | Pages | 372-5 |
| PubMed ID | 16391561 | Mgi Jnum | J:129232 |
| Mgi Id | MGI:3768904 | Doi | 10.1038/sj.ejhg.5201556 |
| Citation | Tsai MS, et al. (2006) Abolishing Trp53-dependent apoptosis does not benefit spinal muscular atrophy model mice. Eur J Hum Genet 14(3):372-5 |
| abstractText | Spinal muscular atrophy (SMA) is the most common genetic motoneuron degenerative disorder, but the mechanism(s) of motoneuron death is unclear. Previously, a direct interaction between tumor-suppressive TP53 protein and the SMA determinant gene product, survival motor neuron protein, was identified and therefore it has been suggested that a mechanism of TP53-dependent apoptosis plays an important role in motoneuron degeneration in SMA. We used our SMA model mice, generated by a combination of knockout and transgenic techniques, to decipher the role of TP53 protein in the motoneuron degeneration in SMA. We detected a significant increase of Trp53 expression in the spinal cord of SMA-like mice compared to their normal littermates. After crossing SMA-like mice with Trp53 knockout mice, the progeny Trp53-deficient SMA-like mice did not show milder disease severity or longer lifespan compared to SMA littermates with wild-type Trp53 genes. Our studies provide in vivo evidence indicating that Trp53-dependent apoptosis does not play a crucial role in motoneuron degeneration in SMA-like mice. European Journal of Human Genetics (2006) 14, 372-375. doi:10.1038/sj.ejhg.5201556; published online 4 January 2006. |
