title: SMN is required for the maintenance of embryonic stem cells and neuronal differentiation in mice.
abstract: Survival motor neuron (SMN) is the determining factor in spinal muscular atrophy, the most common genetic cause of childhood mortality. We have previously found that SMN regulates stem cell division, proliferation and differentiation in Drosophila. However, it is unknown whether a similar effect exists in vertebrates. Here, we show that SMN is enriched in highly proliferative embryonic stem cells (ESCs) in mice and reduction of SMN impairs the pluripotency of ESCs. Moreover, we find that SMN reduction activates ERK signaling and affects neuronal differentiation in vitro. Teratomas with reduced SMN grow more slowly and show weaker signals of neuronal differentiation than those with a normal level of SMN. Finally, we show that over-expression of SMN is protective for ESCs from retinoic acid-induced differentiation. Taken together, our results suggest that SMN plays a role in the maintenance of pluripotent ESCs and neuronal differentiation in mice.
authors.affiliation: Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, Taipei, 106, Taiwan, ROC.
authors.forename: Wei-Fang
authors.initials: WF
authors.lastname: Chang
authors.forename: Jie
authors.initials: J
authors.lastname: Xu
authors.forename: Chia-Chun
authors.initials: CC
authors.lastname: Chang
authors.forename: Shang-Hsun
authors.initials: SH
authors.lastname: Yang
authors.forename: Hsin-Yang
authors.initials: HY
authors.lastname: Li
authors.forename: Hsiu Mei
authors.initials: HM
authors.lastname: Hsieh-Li
authors.forename: Mong-Hsun
authors.initials: MH
authors.lastname: Tsai
authors.forename: Shinn-Chih
authors.initials: SC
authors.lastname: Wu
authors.forename: Winston T K
authors.initials: WT
authors.lastname: Cheng
authors.forename: Ji-Long
authors.initials: JL
authors.lastname: Liu
authors.forename: Li-Ying
authors.initials: LY
authors.lastname: Sung
mesh_terms: D000818:Animals; D002454:Cell Differentiation / Q000235:genetics; D005260:Female; D020935:MAP Kinase Signaling System / Q000235:genetics; D008297:Male; D051379:Mice; D008807:Mice, Inbred BALB C; D000066450:Mouse Embryonic Stem Cells / Q000166:cytology / Q000378:metabolism / Q000502:physiology*; D009474:Neurons / Q000166:cytology* / Q000378:metabolism / Q000502:physiology*; D055533:Survival of Motor Neuron 1 Protein / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D013724:Teratoma / Q000235:genetics / Q000473:pathology
chemical_list: C526472:Smn1 protein, mouse; D055533:Survival of Motor Neuron 1 Protein