Abstract
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).
Copyright © 2013 Elsevier Inc. All rights reserved.
Publication types
-
Research Support, N.I.H., Extramural
-
Research Support, Non-U.S. Gov't
-
Research Support, U.S. Gov't, Non-P.H.S.
MeSH terms
-
Animals
-
Animals, Newborn
-
Base Sequence
-
Cells, Cultured
-
Cerebral Cortex / embryology*
-
Cerebral Cortex / growth & development
-
Corpus Striatum / embryology*
-
Corpus Striatum / growth & development
-
Gene Expression Regulation, Developmental
-
Genes, Homeobox / genetics
-
Homeodomain Proteins / biosynthesis*
-
Homeodomain Proteins / genetics
-
Interneurons / physiology*
-
Mice
-
Mice, Knockout
-
Mice, Transgenic
-
Molecular Sequence Data
-
Neurogenesis / physiology
-
Repressor Proteins / biosynthesis*
-
Repressor Proteins / genetics
-
Transcription Factors / biosynthesis*
-
Transcription Factors / genetics
-
Zinc Finger E-box Binding Homeobox 2
Substances
-
Distal-less homeobox proteins
-
Homeodomain Proteins
-
Repressor Proteins
-
Transcription Factors
-
ZEB2 protein, mouse
-
Zinc Finger E-box Binding Homeobox 2