caa a22 4500 60620198X CHVBK 20210128100951.0 cr unu---uuuuu 210128e20150501xx s 000 0 eng 10.1007/s00429-014-0743-7 doi (NATIONALLICENCE)springer-10.1007/s00429-014-0743-7 SMN is required for the maintenance of embryonic stem cells and neuronal differentiation in mice [Elektronische Daten] [Wei-Fang Chang, Jie Xu, Chia-Chun Chang, Shang-Hsun Yang, Hsin-Yang Li, Hsiu Hsieh-Li, Mong-Hsun Tsai, Shinn-Chih Wu, Winston Cheng, Ji-Long Liu, Li-Ying Sung] 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. Springer-Verlag Berlin Heidelberg, 2014 Survival motor neuron (SMN) nationallicence Embryonic stem cell (ESC) nationallicence ERK signaling nationallicence Neuronal differentiation nationallicence Chang Wei-Fang Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut Xu Jie Center for Advanced Models for Translational Sciences and Therapeutics, University of Michigan Medical Center, Ann Arbor, MI, USA aut Chang Chia-Chun Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut Yang Shang-Hsun Department of Physiology, National Cheng Kung University Medical College, 701, Tainan, Taiwan, ROC aut Li Hsin-Yang Division of Reproductive Endocrinology, Department of Obstetrics and Gynecology, Taipei Veterans General Hospital, 201, Taipei, Taiwan, ROC aut Hsieh-Li Hsiu Department of Life Science, National Taiwan Normal University, 116, Taipei, Taiwan, ROC aut Tsai Mong-Hsun Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut Wu Shinn-Chih Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut Cheng Winston Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut Liu Ji-Long Medical Research Council Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, OX1 3PT, Oxford, UK aut Sung Li-Ying Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut Brain Structure and Function Springer Berlin Heidelberg 220/3(2015-05-01), 1539-1553 1863-2653 220:3<1539 2015 220 429 https://doi.org/10.1007/s00429-014-0743-7 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s00429-014-0743-7 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Chang Wei-Fang Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Xu Jie Center for Advanced Models for Translational Sciences and Therapeutics, University of Michigan Medical Center, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Chang Chia-Chun Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Yang Shang-Hsun Department of Physiology, National Cheng Kung University Medical College, 701, Tainan, Taiwan, ROC aut NATIONALLICENCE 700 1- Li Hsin-Yang Division of Reproductive Endocrinology, Department of Obstetrics and Gynecology, Taipei Veterans General Hospital, 201, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Hsieh-Li Hsiu Department of Life Science, National Taiwan Normal University, 116, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Tsai Mong-Hsun Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Wu Shinn-Chih Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Cheng Winston Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut NATIONALLICENCE 700 1- Liu Ji-Long Medical Research Council Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, OX1 3PT, Oxford, UK aut NATIONALLICENCE 700 1- Sung Li-Ying Institute of Biotechnology, National Taiwan University, No. 81, Chang-Xiang St., Da-an District, 106, Taipei, Taiwan, ROC aut NATIONALLICENCE 773 0- Brain Structure and Function Springer Berlin Heidelberg 220/3(2015-05-01), 1539-1553 1863-2653 220:3<1539 2015 220 429