Abolishing Bax-Dependent Apoptosis Shows Beneficial Effects on Spinal Muscular Atrophy Model Mice

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

研究成果: 雜誌貢獻期刊論文同行評審

37 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 degeneration is unclear. We previously generated SMA model mice, which genotypically and phenotypically mimicked human SMA patients, by a combination of knockout and transgenic techniques. Here, we used these SMA model mice to decipher the apoptotic mechanism(s) involved in SMA motoneuron degeneration. We found a significant increase in proapoptotic Bax expression in the spinal cords of SMA mice in comparison with their wild-type littermates. After crossing SMA mice with Bax knockout mice, we produced in vivo evidence indicating that Bax protein plays an important role in the degeneration of SMA spinal motoneurons. Progeny Bax-deficient SMA mice showed milder disease severity, longer life spans, and significant increases in spinal motoneuron densities compared to SMA littermates with wild-type Bax genes. Our results strongly suggest that suppression of Bax-involved apoptosis has the potential for amelioration of SMA.

原文英語
頁(從 - 到)1149-1155
頁數7
期刊Molecular Therapy
13
發行號6
DOIs
出版狀態已發佈 - 2006 六月

ASJC Scopus subject areas

  • 分子醫學
  • 分子生物學
  • 遺傳學
  • 藥理
  • 藥物發現

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