TY - JOUR
T1 - Dlx1&2-Dependent Expression of Zfhx1b (Sip1, Zeb2) Regulates the Fate Switch between Cortical and Striatal Interneurons
AU - McKinsey, Gabriel L.
AU - Lindtner, Susan
AU - Trzcinski, Brett
AU - Visel, Axel
AU - Pennacchio, Len A.
AU - Huylebroeck, Danny
AU - Higashi, Yujiro
AU - Rubenstein, John L.R.
N1 - Funding Information:
This work was supported by the research grants to J.L.R.R. from Nina Ireland, Weston Havens Foundation, Genentech, NIMH R37 MH049428 and R01 MH081880; to G.L.M. from Predoctoral Training in Neurobiology T32 GM007449; to L.A.P. and A.V. from NINDS R01NS062859A and NHGRI R01HG003988.; to G.L.M. from Predoctoral Training in Neurobiology T32 GM007449. L.A.P. and A.V. conducted research at the E.O. Lawrence Berkeley National Laboratory, performed under DOE DE-AC02-05CH11231, University of California. D.H. is funded by Queen Elisabeth Medical Foundation (1113), Scientific Research-Flanders (G.0954.11N), Research Council University of Leuven GOA, InfraMouse Hercules, and Belspo IUAP7/07. We thank Professor Melinda Duncan at the University of Delaware for providing Zfhx1b F/+ mice.
PY - 2013/1/9
Y1 - 2013/1/9
N2 - Mammalian pallial (cortical and hippocampal) and striatal interneurons are both generated in the embryonic subpallium, including the medial ganglionic eminence (MGE). Herein we demonstrate that the Zfhx1b (Sip1, Zeb2) zinc finger homeobox gene is required in the MGE, directly downstream of Dlx1&2, to generate cortical interneurons that express Cxcr7, MafB, and cMaf. In its absence, Nkx2-1 expression is not repressed, and cells that ordinarily would become cortical interneurons appear to transform toward a subtype of GABAergic striatal interneurons. These results show that Zfhx1b is required to generate cortical interneurons, and suggest a mechanism for the epilepsy observed in humans with Zfhx1b mutations (Mowat-Wilson syndrome).
AB - Mammalian pallial (cortical and hippocampal) and striatal interneurons are both generated in the embryonic subpallium, including the medial ganglionic eminence (MGE). Herein we demonstrate that the Zfhx1b (Sip1, Zeb2) zinc finger homeobox gene is required in the MGE, directly downstream of Dlx1&2, to generate cortical interneurons that express Cxcr7, MafB, and cMaf. In its absence, Nkx2-1 expression is not repressed, and cells that ordinarily would become cortical interneurons appear to transform toward a subtype of GABAergic striatal interneurons. These results show that Zfhx1b is required to generate cortical interneurons, and suggest a mechanism for the epilepsy observed in humans with Zfhx1b mutations (Mowat-Wilson syndrome).
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U2 - 10.1016/j.neuron.2012.11.035
DO - 10.1016/j.neuron.2012.11.035
M3 - Article
C2 - 23312518
AN - SCOPUS:84872193366
SN - 0896-6273
VL - 77
SP - 83
EP - 98
JO - Neuron
JF - Neuron
IS - 1
ER -