Survival Motor Neuron Enhances Pluripotent Gene Expression and Facilitates Cell Reprogramming.
Journal
Stem cells and development
Journal Volume
31
Journal Issue
21-22
Start Page
696
End Page
705
ISSN
1557-8534
Date Issued
2022-11
Author(s)
Chang, Wei-Fang
Lin, Tzu-Ying
Peng, Min
Xu, Jie
Hsieh-Li, Hsiu-Mei
Liu, Ji-Long
Abstract
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 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.
Subjects
induced pluripotent stem cells
neuronal differentiation
somatic cell reprogramming
spinal muscular atrophy
survival motor neuron
SDGs
Publisher
Liebert
Type
journal article