The DOI matches its Crossref record; 19 of the 19 references it cites were found in a registry or an index.
Rett Syndrome Astrocytes Are Abnormal and Spread MeCP2 Deficiency through Gap Junctions
Open accessAbstract
MECP2 , an X-linked gene encoding the epigenetic factor methyl-CpG-binding protein-2, is mutated in Rett syndrome (RTT) and aberrantly expressed in autism. Most children affected by RTT are heterozygous Mecp2 −/+ females whose brain function is impaired postnatally due to MeCP2 deficiency. While prior functional investigations of MeCP2 have focused exclusively on neurons and have concluded the absence of MeCP2 in astrocytes, here we report that astrocytes express MeCP2, and MeCP2 deficiency in astrocytes causes significant abnormalities in BDNF regulation, cytokine production, and neuronal dendritic induction, effects that may contribute to abnormal neurodevelopment. In addition, we show that the MeCP2 deficiency state can progressively spread at least in part via gap junction communications between mosaic Mecp2 −/+ astrocytes in a novel non-cell-autonomous mechanism. This mechanism may lead to the pronounced loss of MeCP2 observed selectively in astrocytes in mouse Mecp2 −/+ brain, which is coincident with phenotypic regression characteristic of RTT. Our results suggest that astrocytes are viable therapeutic targets for RTT and perhaps regressive forms of autism.
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MeCP2, a Key Contributor to Neurological Disease, Activates and Represses Transcription
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DNA Methylation-Related Chromatin Remodeling in Activity-Dependent Bdnf Gene Regulation
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Astrocytes are active players in cerebral innate immunity
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Astrocytic Purinergic Signaling Coordinates Synaptic Networks
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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
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Derepression of BDNF Transcription Involves Calcium-Dependent Phosphorylation of MeCP2
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Reversal of Neurological Defects in a Mouse Model of Rett Syndrome
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Cited by
Marchetto, M. C. N., Carromeu, C., Acab, A., Yu, D., Yeo, G. W., Mu, Y., Chen, G., Gage, F. H., & Muotri, A. R. (2010) · Cell · doi:10.1016/j.cell.2010.10.016 · Cited by 1,329
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
Zoghbi, H. Y., & Bear, M. F. (2012) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a009886 · Cited by 797
Wild-type microglia arrest pathology in a mouse model of Rett syndrome
Derecki, N. C., Cronk, J. C., Lu, Z., Xu, E., Abbott, S. B. G., Guyenet, P. G., & Kipnis, J. (2012) · Nature · doi:10.1038/nature10907 · Cited by 607
The Indispensable Roles of Microglia and Astrocytes during Brain Development
Reemst, K., Noctor, S. C., Lucassen, P. J., & Hol, E. M. (2016) · Frontiers in Human Neuroscience · doi:10.3389/fnhum.2016.00566 · Cited by 516
Metadata
- Authors
- Maezawa, I., Swanberg, S., Harvey, D., LaSalle, J. M., & Jin, L.-W.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2009-04-22
- Volume
- 29
- Issue
- 16
- Pages
- 5051-5061
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W1994210488
- Cited by
- 285
- References
- 19
- FWCI
- 14.97
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Maezawa, I., Swanberg, S., Harvey, D., LaSalle, J. M., & Jin, L.-W. (2009). Rett Syndrome Astrocytes Are Abnormal and Spread MeCP2 Deficiency through Gap Junctions. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.0324-09.2009
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
