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The Journal of Neuroscience, May 28, 2008, 28(22):5817-5826; doi:10.1523/JNEUROSCI.0853-08.2008

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Neurobiology of Disease
GPR56 Regulates Pial Basement Membrane Integrity and Cortical Lamination

Shihong Li,¹ Zhaohui Jin,^1 * Samir Koirala,^2 * Lihong Bu,^1 * Lei Xu,⁴ Richard O. Hynes,⁴ Christopher A. Walsh,³ Gabriel Corfas,² and Xianhua Piao¹

¹Division of Newborn Medicine, Department of Medicine, ²Neurobiology Program, Children's Hospital Boston, Harvard Medical School, ³Department of Neurology, Howard Hughes Medical Institute, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02115, and ⁴Howard Hughes Medical Institute, Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139

Correspondence should be addressed to Dr. Xianhua Piao, 300 Longwood Avenue, Boston, MA 02115. Email: xianhua.piao{at}childrens.harvard.edu

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.

Key words: GPR56; bilateral frontoparietal polymicrogyria; BFPP; basement membrane; cobblestone cortex; cortical lamination

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Received Feb. 26, 2008; revised April 15, 2008; accepted April 20, 2008.

Correspondence should be addressed to Dr. Xianhua Piao, 300 Longwood Avenue, Boston, MA 02115. Email: xianhua.piao{at}childrens.harvard.edu

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