@article {309997,
	title = {Mutations in genes encoding the cadherin receptor-ligand pair DCHS1 and FAT4 disrupt cerebral cortical development},
	journal = {Nat Genet},
	volume = {45},
	year = {2013},
	month = {11/2013},
	pages = {1300 - 1308},
	abstract = {<p>The regulated proliferation and differentiation of neural stem cells before the generation and migration of neurons in the cerebral cortex are central aspects of mammalian development. Periventricular neuronal heterotopia, a specific form of mislocalization of cortical neurons, can arise from neuronal progenitors that fail to negotiate aspects of these developmental processes. Here we show that mutations in genes encoding the receptor-ligand cadherin pair DCHS1 and FAT4 lead to a recessive syndrome in humans that includes periventricular neuronal heterotopia. Reducing the expression of Dchs1 or Fat4 within mouse embryonic neuroepithelium increased progenitor cell numbers and reduced their differentiation into neurons, resulting in the heterotopic accumulation of cells below the neuronal layers in the neocortex, reminiscent of the human phenotype. These effects were countered by concurrent knockdown of Yap, a transcriptional effector of the Hippo signaling pathway. These findings implicate Dchs1 and Fat4 upstream of Yap as key regulators of mammalian neurogenesis.</p>
},
	isbn = {1061-4036},
	url = {http://dx.doi.org/10.1038/ng.2765},
	author = {Cappello, S. and Gray, M. J. and Badouel, C. and Lange, S. and Einsiedler, M. and Srour, M. and Chitayat, D. and Hamdan, F. F. and Jenkins, Z. A. and Morgan, T. and Preitner, N. and Uster, T. and Thomas, J. and Shannon, P. and Morrison, V. and Di Donato, N. and Van Maldergem, L. and Neuhann, T. and Newbury-Ecob, R. and Swinkells, M. and Terhal, P. and Wilson, L. C. and Zwijnenburg, P. J. G. and Sutherland-Smith, A. J. and Black, M. A. and Markie, D. and Michaud, J. L. and Simpson, M. A. and Mansour, S. and McNeill, H. and Gotz, M. and Robertson, S. P.}
}
