Survival Motor Neuron Enhances Pluripotent Gene Expression and Facilitates Cell Reprogramming

Wei Fang Chang, Tzu Ying Lin, Min Peng, Chia Chun Chang, Jie Xu, Hsiu Mei Hsieh-Li, Ji Long Liu, Li Ying Sung*


研究成果: 雜誌貢獻期刊論文同行評審

2 引文 斯高帕斯(Scopus)


Survival motor neuron (SMN) plays important roles in snRNP assembly and mRNA splicing. Deficiency of SMN causes spinal muscular atrophy (SMA), a leading genetic disease causing childhood mortality. Previous studies have shown that SMN regulates stem cell self-renewal and pluripotency in Drosophila and mouse and is abundantly expressed in mouse embryonic stem cells. However, whether SMN is required for establishment of pluripotency is unclear. In this study, we show that SMN is gradually upregulated in preimplantation mouse embryos and cultured cells undergoing cell reprogramming. Ectopic expression of SMN increased cell reprogramming efficiency, whereas knockdown of SMN impeded induced pluripotent stem cell (iPSC) colony formation. iPSCs could be derived from SMA model mice, but impairment in differentiation capacity may be present. The ectopic overexpression of SMN in iPSCs can upregulate the expression levels of some pluripotent genes and restore the neuronal differentiation capacity of SMA-iPSCs. Taken together, our findings not only demonstrate the functional relevance of SMN in establishment of cell pluripotency but also propose its potential application in facilitating iPSC derivation.

頁(從 - 到)696-705
期刊Stem Cells and Development
出版狀態已發佈 - 2022 11月

ASJC Scopus subject areas

  • 血液學
  • 發展生物學
  • 細胞生物學


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