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Title: Cell cycle inhibitors protect motor neurons in an organoid model of Spinal Muscular Atrophy
Authors: Hor, Jin Hui
Soh, Eunice Shi-Yi
Tan, Li Yi
Lim, Valerie Jing Wen
Ho, Beatrice Xuan
Fan, Yong
Soh, Boon-Seng
Ng, Shi-Yan
Munirah Mohamad Santosa
Keywords: DRNTU::Science::Biological sciences
Cell Cycle Inhibitors
Spinal Muscular Atrophy
Issue Date: 2018
Source: Hor, 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/s41419-018-1081-0
Series/Report no.: Cell Death & Disease
Abstract: Spinal 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.
DOI: 10.1038/s41419-018-1081-0
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
Fulltext Permission: open
Fulltext Availability: With Fulltext
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