Seth, E.A. and Lee, H.-C. and bin Md Yusof, H.H. and Nordin, N. and Cheah, Y.K. and Wei Ho, E.T. and Ling, K.-H. and Cheah, P.-S. (2020) Phenotype microarrays reveal metabolic dysregulations of neurospheres derived from embryonic Ts1Cje mouse model of down syndrome. PLoS ONE, 15 (7 July). ISSN 19326203
Full text not available from this repository.Abstract
Down syndrome (DS), is the most common cause of intellectual disability, and is characterized by defective neurogenesis during perinatal development. To identify metabolic aberrations in early neurogenesis, we profiled neurospheres derived from the embryonic brain of Ts1Cje, a mouse model of Down syndrome. High-throughput phenotypic microarray revealed a significant decrease in utilisation of 17 out of 367 substrates and significantly higher utilisation of 6 substrates in the Ts1Cje neurospheres compared to controls. Specifically, Ts1Cje neurospheres were less efficient in the utilisation of glucose-6-phosphate suggesting a dysregulation in the energy-producing pathway. T Cje neurospheres were significantly smaller in diameter than the controls. Subsequent preliminary study on supplementation with 6-phosphogluconic acid, an intermediate of glucose-6-phosphate metabolism, was able to rescue the Ts1Cje neurosphere size. This study confirmed the perturbed pentose phosphate pathway, contributing to defects observed in Ts1Cje neurospheres. We show for the first time that this comprehensive energetic assay platform facilitates the metabolic characterisation of Ts1Cje cells and confirmed their distinguishable metabolic profiles compared to the controls. Copyright: © 2020 Seth et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Item Type: | Article |
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Additional Information: | cited By 5 |
Uncontrolled Keywords: | glucose 6 phosphate; pentose phosphate; phosphogluconate dehydrogenase, animal cell; animal experiment; animal model; animal tissue; Article; brain metabolism; cell proliferation; cell size; controlled study; Down syndrome; embryo; energy metabolism; enzyme metabolism; female; high throughput analysis; male; metabolic disorder; mouse; nervous system development; nonhuman; phenotype; signal transduction; animal; brain; C57BL mouse; Down syndrome; embryology; metabolism; microarray analysis; nerve cell; nervous system development; pathology; phenotype, Animals; Brain; Down Syndrome; Female; Mice; Mice, Inbred C57BL; Microarray Analysis; Neurogenesis; Neurons; Phenotype |
Depositing User: | Mr Ahmad Suhairi UTP |
Date Deposited: | 10 Nov 2023 03:27 |
Last Modified: | 10 Nov 2023 03:27 |
URI: | https://khub.utp.edu.my/scholars/id/eprint/12940 |