QUICK SEARCH:   [advanced] Author: Keyword(s):
Home     Help     Feedback     Subscriptions     Archive     Search     Table of Contents

First published online 3 October 2007
doi: 10.1242/dev.004325

This Article Figures Only Full Text Full Text (PDF) Supplementary Material All Versions of this Article: dev.004325v1 134/21/3789    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Fernandes, M. Articles by Hébert, J. M. Search for Related Content PubMed PubMed Citation Articles by Fernandes, M. Articles by Hébert, J. M. Social Bookmarking                         What's this?

Mutations in the BMP pathway in mice support the existence of two molecular classes of holoprosencephaly

¹ Departments of Neuroscience and Molecular Genetics, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
² Developmental Biology Program, Sloan-Kettering Institute, New York, NY 10021, USA.
³ Department of Biological Sciences, Stanford University, Stanford, CA, USA.

^* Author for correspondence (e-mail: jhebert{at}aecom.yu.edu)

Accepted 29 August 2007

SUMMARY

Holoprosencephaly (HPE) is a devastating forebrain abnormality with a range of morphological defects characterized by loss of midline tissue. In the telencephalon, the embryonic precursor of the cerebral hemispheres, specialized cell types form a midline that separates the hemispheres. In the present study, deletion of the BMP receptor genes, Bmpr1b and Bmpr1a, in the mouse telencephalon results in a loss of all dorsal midline cell types without affecting the specification of cortical and ventral precursors. In the holoprosencephalic Shh^-/- mutant, by contrast, ventral patterning is disrupted, whereas the dorsal midline initially forms. This suggests that two separate developmental mechanisms can underlie the ontogeny of HPE. The Bmpr1a;Bmpr1b mutant provides a model for a subclass of HPE in humans: midline inter-hemispheric HPE.

Key words: Telencephalon, Dorsal midline, Choroid plexus, Cortical hem, Holoprosencephaly, BMP, SHH

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				R. Dobrowolski, G. Hertig, H. Lechner, P. Worsdorfer, V. Wulf, N. Dicke, D. Eckert, R. Bauer, H. Schorle, and K. Willecke Loss of connexin43-mediated gap junctional coupling in the mesenchyme of limb buds leads to altered expression of morphogens in mice Hum. Mol. Genet., August 1, 2009; 18(15): 2899 - 2911. [Abstract] [Full Text] [PDF]

				H. Paek, G. Gutin, and J. M. Hebert FGF signaling is strictly required to maintain early telencephalic precursor cell survival Development, July 15, 2009; 136(14): 2457 - 2465. [Abstract] [Full Text] [PDF]

				D. Maurus and W. A. Harris Zic-associated holoprosencephaly: zebrafish Zic1 controls midline formation and forebrain patterning by regulating Nodal, Hedgehog, and retinoic acid signaling Genes & Dev., June 15, 2009; 23(12): 1461 - 1473. [Abstract] [Full Text] [PDF]

				I. Imayoshi, T. Shimogori, T. Ohtsuka, and R. Kageyama Hes genes and neurogenin regulate non-neural versus neural fate specification in the dorsal telencephalic midline Development, August 1, 2008; 135(15): 2531 - 2541. [Abstract] [Full Text] [PDF]

				M. S. Brill, M. Snapyan, H. Wohlfrom, J. Ninkovic, M. Jawerka, G. S. Mastick, R. Ashery-Padan, A. Saghatelyan, B. Berninger, and M. Gotz A Dlx2- and Pax6-Dependent Transcriptional Code for Periglomerular Neuron Specification in the Adult Olfactory Bulb J. Neurosci., June 18, 2008; 28(25): 6439 - 6452. [Abstract] [Full Text] [PDF]

				B. G. Rash and E. A. Grove Patterning the Dorsal Telencephalon: A Role for Sonic Hedgehog? J. Neurosci., October 24, 2007; 27(43): 11595 - 11603. [Abstract] [Full Text] [PDF]