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GPR56 Regulates Pial Basement Membrane Integrity and Cortical Lamination
Open accessAbstract
GPR56 is a member of the family of adhesion G-protein-coupled receptors that have a large extracellular region containing a GPS (G-protein proteolytic site) domain. Loss-of-function mutations in the GPR56 gene cause a specific human brain malformation called bilateral frontoparietal polymicrogyria (BFPP). BFPP is a radiological diagnosis and its histopathology remains unclear. This study demonstrates that loss of the mouse Gpr56 gene leads to neuronal ectopia in the cerebral cortex, a cobblestone-like cortical malformation. There are four crucial events in the development of cobblestone cortex, namely defective pial basement membrane (BM), abnormal anchorage of radial glial endfeet, mislocalized Cajal–Retzius cells, and neuronal overmigration. By detailed time course analysis, we reveal that the leading causal events are likely the breaches in the pial BM. We show further that GPR56 is present in abundance in radial glial endfeet. Furthermore, a putative ligand of GPR56 is localized in the marginal zone or overlying extracellular matrix. These observations provide compelling evidence that GPR56 functions in regulating pial BM integrity during cortical development.
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In the citing paper
“GPR56 encodes an adhesion heterotrimeric guanine nucleotide–binding protein (G protein)–coupled receptor that is highly expressed in cortical progenitors (7, 16) and binds extracellular matrix proteins (17).”
From Evolutionarily Dynamic Alternative Splicing of GPR56 Regulates Regional Cerebral Cortical Patterning (2014) · cites this paper as [16]
In the citing paper
“GPR56 likely influences progenitor proliferation by stabilizing the basal process of radial neuroepithelial progenitors”
From Evolutionarily Dynamic Alternative Splicing of GPR56 Regulates Regional Cerebral Cortical Patterning (2014) · cites this paper as [16]
In the citing paper
“GPR56 protein is most highly expressed in progenitors in the ventricular and subventricular zones during neurogenesis in mice (16, 18).”
From Evolutionarily Dynamic Alternative Splicing of GPR56 Regulates Regional Cerebral Cortical Patterning (2014) · cites this paper as [16]
In the citing paper
“Loss of GPR56 disrupts radial glia and causes breaches in the pial basement membrane, through which some neurons over-migrate (9, 16).”
From Evolutionarily Dynamic Alternative Splicing of GPR56 Regulates Regional Cerebral Cortical Patterning (2014) · cites this paper as [16]
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Post-translational disruption of dystroglycan–ligand interactions in congenital muscular dystrophies
Michele, D. E., Barresi, R., Kanagawa, M., Saito, F., Cohn, R. D., Satz, J. S., Dollar, J., Nishino, I., Kelley, R. I., Somer, H., et al. (2002) · Nature · doi:10.1038/nature00837 · Cited by 779
Malatesta, P., Hack, M. A., Hartfuss, E., Kettenmann, H., Klinkert, W. E. F., Kirchhoff, F., & Götz, M. (2003) · Neuron · doi:10.1016/s0896-6273(03)00116-8 · Cited by 771
Tissir, F., & Goffinet, A. M. (2003) · Nature reviews. Neuroscience · doi:10.1038/nrn1113 · Cited by 767
Yoshida, A., Kobayashi, K., Manya, H., Taniguchi, K., Kano, H., Mizuno, M., Inazu, T., Mitsuhashi, H., Takahashi, S., Takeuchi, M., et al. (2001) · Developmental Cell · doi:10.1016/s1534-5807(01)00070-3 · Cited by 706
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Costell, M., Gustafsson, E., Aszódi, A., Mörgelin, M., Bloch, W., Hunziker, E. B., Addicks, K., Timpl, R., & Fässler, R. (1999) · The Journal of Cell Biology · doi:10.1083/jcb.147.5.1109 · Cited by 672
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Nieto, M., Monuki, E. S., Tang, H., Imitola, J., Haubst, N., Khoury, S. J., Cunningham, J., Götz, M., & Walsh, C. A. (2004) · The Journal of Comparative Neurology · doi:10.1002/cne.20322 · Cited by 569
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Metadata
- Authors
- Li, S., Jin, Z., Koirala, S., Bu, L., Xu, L., Hynes, R. O., Walsh, C. A., Corfas, G., & Piao, X.
- Affiliations
- Boston Children's Hospital (US)
- Harvard University (US)
- Howard Hughes Medical Institute (US)
- Beth Israel Deaconess Medical Center (US)
- ORCID
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2008-05-28
- Volume
- 28
- Issue
- 22
- Pages
- 5817-5826
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- PMC
- PMC2504715
- OpenAlex
- W2065631407
- Cited by
- 241
- References
- 37
- Citations by year
- 2026: 10 · 2025: 6 · 2024: 8 · 2023: 11 · 2022: 12 · 2021: 7 · 2020: 16 · 2019: 15 · 2018: 8 · 2017: 9 · 2016: 16 · 2015: 19 · 2014: 16 · 2013: 17 · 2012: 16
- FWCI
- 5.39
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Neuroscience (publishedVersion, cc-by-nc-sa)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Charles H. Hood Foundation
- Flight Attendant Medical Research Institute
- National Institutes of Health
- Genentech
- National Institute of Neurological Disorders and Stroke (R01 NS35884, R01NS057536, K08NS045762, T32NS007484, R01NS035884)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (P30HD018655, P30HD-18655)
- Topics
- Life Sciences › Neuroscience › Developmental Neuroscience › Neurogenesis and neuroplasticity mechanisms
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Keywords
- GPR56, adhesion G protein-coupled receptors, cortical lamination, Cajal-Retzius cells, cortical development, extracellular matrix
- MeSH terms
- Animals, Animals, Newborn, Bacterial Outer Membrane Proteins, Basement Membrane, Bromodeoxyuridine, Cell Movement, Cerebral Cortex, Embryo, Mammalian, Mutation, Neuroglia, Neurons, Pia Mater, Stem Cells, Tubulin, Mice, Knockout, Gene Expression Regulation, Developmental, ATP-Binding Cassette Transporters, Lamins, Receptors, G-Protein-Coupled, Mice
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