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*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Citations (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.

Original languageEnglish
Pages (from-to)696-705
Number of pages10
JournalStem Cells and Development
Issue number21-22
Publication statusPublished - 2022 Nov


  • induced pluripotent stem cells
  • neuronal differentiation
  • somatic cell reprogramming
  • spinal muscular atrophy
  • survival motor neuron

ASJC Scopus subject areas

  • Hematology
  • Developmental Biology
  • Cell Biology


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