Abolishing Trp53-dependent apoptosis does not benefit spinal muscular atrophy model mice

Ming S. Tsai, Yung T. Chiu, Sue H. Wang, Hsiu M. Hsieh-Li, Hung Li*

*此作品的通信作者

研究成果: 雜誌貢獻期刊論文同行評審

9 引文 斯高帕斯(Scopus)

摘要

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.

原文英語
頁(從 - 到)372-375
頁數4
期刊European Journal of Human Genetics
14
發行號3
DOIs
出版狀態已發佈 - 2006 三月

ASJC Scopus subject areas

  • 遺傳學
  • 遺傳學(臨床)

指紋

深入研究「Abolishing Trp53-dependent apoptosis does not benefit spinal muscular atrophy model mice」主題。共同形成了獨特的指紋。

引用此