The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.
Mutant astrocytes differentiated from Rett syndrome patients-specific iPSCs have adverse effects on wild-type neurons
Open accessAbstract
The disease mechanism of Rett syndrome (RTT) is not well understood. Studies in RTT mouse models have suggested a non-cell-autonomous role for astrocytes in RTT pathogenesis. However, it is not clear whether this is also true for human RTT astrocytes. To establish an in vitro human RTT model, we previously generated isogenic induced pluripotent stem cell (iPSC) lines from several RTT patients carrying different disease-causing mutations. Here, we show that these RTT iPSC lines can be efficiently differentiated into astroglial progenitors and glial fibrillary acidic protein-expressing (GFAP(+)) astrocytes that maintain isogenic status, that mutant RTT astrocytes carrying three different RTT mutations and their conditioned media have adverse effects on the morphology and function of wild-type neurons and that the glial effect on neuronal morphology is independent of the intrinsic neuronal deficit in mutant neurons. Moreover, we show that both insulin-like growth factor 1 (IGF-1) and GPE (a peptide containing the first 3 amino acids of IGF-1) are able to partially rescue the neuronal deficits caused by mutant RTT astrocytes. Our findings confirm the critical glial contribution to RTT pathology, reveal potential cellular targets of IGF-1 therapy and further validate patient-specific iPSCs and their derivatives as valuable tools to study RTT disease mechanism.
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Cites
Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2
Amir, R. E., Van den Veyver, I. B., Wan, M., Tran, C. Q., Francke, U., & Zoghbi, H. Y. (1999) · Nature Genetics · doi:10.1038/13810 · Cited by 4,990
Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice
Chen, R. Z., Akbarian, S., Tudor, M., & Jaenisch, R. (2001) · Nature Genetics · doi:10.1038/85906 · Cited by 1,275
Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes
Chao, H.-T., Chen, H., Samaco, R. C., Xue, M., Chahrour, M., Yoo, J., Neul, J. L., Gong, S., Lu, H.-C., Heintz, N., et al. (2010) · Nature · doi:10.1038/nature09582 · Cited by 1,168
Partial reversal of Rett Syndrome-like symptoms in MeCP2 mutant mice
Tropea, D., Giacometti, E., Wilson, N. R., Beard, C., McCurry, C., Fu, D. D., Flannery, R., Jaenisch, R., & Sur, M. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0812394106 · Cited by 593
A role for glia in the progression of Rett’s syndrome
Lioy, D. T., Garg, S. K., Monaghan, C. E., Raber, J., Foust, K. D., Kaspar, B. K., Hirrlinger, P. G., Kirchhoff, F., Bissonnette, J. M., Ballas, N., et al. (2011) · Nature · doi:10.1038/nature10214 · Cited by 496
Non–cell autonomous influence of MeCP2-deficient glia on neuronal dendritic morphology
Ballas, N., Lioy, D. T., Grunseich, C., & Mandel, G. (2009) · Nature Neuroscience · doi:10.1038/nn.2275 · Cited by 466
Kishi, N., & Macklis, J. D. (2004) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2004.07.006 · Cited by 445
Selective dendritic alterations in the cortex of Rett syndrome
Armstrong, D., Dunn, J. K., Antalffy, B., & Trivedi, R. (1995) · Journal of Neuropathology and Experimental Neurology · doi:10.1097/00005072-199503000-00006 · Cited by 378
Johnson, M. A., Weick, J. P., Pearce, R. A., & Zhang, S.-C. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.4562-06.2007 · Cited by 318
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Cited by
Cell Biology of Astrocyte-Synapse Interactions
Allen, N. J., & Eroglu, C. (2017) · Neuron · doi:10.1016/j.neuron.2017.09.056 · Cited by 1,098
Pluripotent stem cells in disease modelling and drug discovery
Avior, Y., Sagi, I., & Benvenisty, N. (2016) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2015.27 · Cited by 592
Mellios, N., Feldman, D. A., Sheridan, S. D., Ip, J. P. K., Kwok, S., Amoah, S. K., Rosen, B., Rodriguez, B. A., Crawford, B., Swaminathan, R., et al. (2018) · Molecular Psychiatry · doi:10.1038/mp.2017.86 · Cited by 292
Functional recovery with recombinant human IGF1 treatment in a mouse model of Rett syndrome
Castro, J., Garcia, R. I., Kwok, S., Banerjee, A., Petravicz, J., Woodson, J., Mellios, N., Tropea, D., & Sur, M. (2014) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1311685111 · Cited by 242
Tchieu, J., Calder, E. L., Guttikonda, S. R., Gutzwiller, E. M., Aromolaran, K. A., Steinbeck, J. A., Goldstein, P. A., & Studer, L. (2019) · Nature Biotechnology · doi:10.1038/s41587-019-0035-0 · Cited by 231
Metadata
- Authors
- Williams, E. C., Zhong, X., Mohamed, A., Li, R., Liu, Y., Dong, Q., Ananiev, G. E., Mok, J. C. C., Lin, B. R., Lu, J., Chiao, C., Cherney, R., Li, H., Zhang, S.-C., & Chang, Q.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2014-01-12
- Volume
- 23
- Issue
- 11
- Pages
- 2968-2980
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2123292451
- Cited by
- 201
- References
- 9
- FWCI
- 15.26
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Williams, E. C., Zhong, X., Mohamed, A., Li, R., Liu, Y., Dong, Q., Ananiev, G. E., Mok, J. C. C., Lin, B. R., Lu, J., Chiao, C., Cherney, R., Li, H., Zhang, S.-C., & Chang, Q. (2014). Mutant astrocytes differentiated from Rett syndrome patients-specific iPSCs have adverse effects on wild-type neurons. Human Molecular Genetics. https://doi.org/10.1093/hmg/ddu008
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