Abstract
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.
Original language | English (US) |
---|---|
Pages (from-to) | 3789-3794 |
Number of pages | 6 |
Journal | Development |
Volume | 134 |
Issue number | 21 |
DOIs | |
State | Published - Nov 2007 |
Keywords
- BMP
- Choroid plexus
- Cortical hem
- Dorsal midline
- Holoprosencephaly
- SHH
- Telencephalon
ASJC Scopus subject areas
- Molecular Biology
- Developmental Biology