Central Roles of the Roof Plate in Telencephalic Development and Holoprosencephaly.
From: Department of Pathology and Laboratory Medicine, University of California Irvine School of Medicine, 92697-4800, USA.
The Journal of neuroscience : the official journal of the Society for Neuroscience
- Publish Date: Jul 2006
- ISSN: 1529-2401
- Volume: 26
- Issue: 29
- Pages: 7640-9
- Medium: Internet
- Language: English
- Citation (JAMA): Cheng Xun, Hsu Ching-mei, Currle D Spencer, et al. Central Roles of the Roof Plate in Telencephalic Development and Holoprosencephaly.. J. Neurosci. Jul 2006;26:7640-9
Abstract
The roof plate is a well known signaling center in CNS development, but its roles in the developing telencephalon and the common holoprosencephaly (HPE) malformation have been uncertain. Using cellular ablations in mice, we show that roof plate cell loss causes failed midline induction and HPE in the dorsal telencephalon. This morphologic phenotype is accompanied by selective deficits in midline gene expression and a reduced activity gradient for bone morphogenetic proteins (Bmps), the major signals produced by the roof plate. In dissociated cells and mutant explants, exogenous Bmp4 is sufficient to mimic roof plate selectivity in midline gene regulation and to rescue roof plate-dependent midline patterning. Previously unrecognized neuroanatomical defects predicted by the mouse model are then confirmed in human HPE patients. These findings establish selective roles for roof plate-dependent Bmp signaling in dorsal telencephalic patterning and HPE and define novel candidate genes for the human disorder.
Mesh Headings (Keywords): Animals, Body Patterning, Bone Morphogenetic Proteins, Cerebral Cortex, Embryo, Mammalian, Embryonic Development, Gene Expression Regulation, Developmental, Holoprosencephaly, Humans, Mice, Mice, Inbred Strains, Nervous System, Neural Tube Defects, Phenotype, Telencephalon, Transcription Factors, Up-Regulation
Check for Full Text / PubMed Unique Identifier (PMID): 16855091
This abstract is part of PubMed, a service of the U.S. National Library of Medicine. PubMed includes more than 17 million citations from MEDLINE and other life science journals for biomedical articles. See Copyright and Disclaimers.
Linked medical terms appearing on this page are added by Healia to help readers find more information and are not part of the original PubMed document.
The data herein was last updated on July 8th, 2008 and may not reflect the most current and accurate data available from NLM.