dc.contributor.authorHor, Jin Hui
dc.contributor.authorSoh, Eunice Shi-Yi
dc.contributor.authorTan, Li Yi
dc.contributor.authorLim, Valerie Jing Wen
dc.contributor.authorMunirah Mohamad Santosa
dc.contributor.authorHo, Beatrice Xuan
dc.contributor.authorFan, Yong
dc.contributor.authorSoh, Boon-Seng
dc.contributor.authorNg, Shi-Yan
dc.identifier.citationHor, J. H., Soh, E. S.-Y., Tan, L. Y., Lim, V. J. W., Munirah Mohamad Santosa, Winanto, . . . Ng, S.-Y. (2018). Cell cycle inhibitors protect motor neurons in an organoid model of Spinal Muscular Atrophy. Cell Death & Disease, 9, 1100-. doi:10.1038/s41419-018-1081-0en_US
dc.description.abstractSpinal Muscular Atrophy (SMA) is caused by genetic mutations in the SMN1 gene, resulting in drastically reduced levels of Survival of Motor Neuron (SMN) protein. Although SMN is ubiquitously expressed, spinal motor neurons are one of the most affected cell types. Previous studies have identified pathways uniquely activated in SMA motor neurons, including a hyperactivated ER stress pathway, neuronal hyperexcitability, and defective spliceosomes. To investigate why motor neurons are more affected than other neural types, we developed a spinal organoid model of SMA. We demonstrate overt motor neuron degeneration in SMA spinal organoids, and this degeneration can be prevented using a small molecule inhibitor of CDK4/6, indicating that spinal organoids are an ideal platform for therapeutic discovery.en_US
dc.description.sponsorshipASTAR (Agency for Sci., Tech. and Research, S’pore)en_US
dc.format.extent12 p.en_US
dc.relation.ispartofseriesCell Death & Diseaseen_US
dc.rights© 2018 The Author(s). This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.en_US
dc.subjectSpinal Muscular Atrophyen_US
dc.subjectCell Cycle Inhibitorsen_US
dc.subjectDRNTU::Science::Biological sciencesen_US
dc.titleCell cycle inhibitors protect motor neurons in an organoid model of Spinal Muscular Atrophyen_US
dc.typeJournal Article
dc.contributor.schoolSchool of Biological Sciencesen_US
dc.description.versionPublished versionen_US

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