LAUSR

Survival motor neuron (SMN) plays important roles in snRNPs assembly and mRNA splicing. Deficiency of SMN causes spinal muscular atrophy (SMA), a leading genetic disease of childhood mortality. Previous studies have shown that SMN regulates stem cell self-renewal and pluripotency in Drosophila and in mouse, and is abundantly expressed in mouse embryonic stem cells (ESCs). However, whether SMN is required for the establishment of pluripotency is unclear. Herein, we show that SMN is gradually upregulated in pre-implantation mouse embryos and cultured cells undergoing cell reprogramming. Ectopic expression of SMN increased the cell reprogramming efficiency, whereas knockdown of SMN impeded iPSC colony formation. iPSCs could be derived from SMA model mice, but certain impairment in differentiation capacity may 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 and the establishment of cell pluripotency, but also propose its potential application in facilitating iPSC derivation.