Tbx1, a DiGeorge Syndrome Candidate Gene, Is Regulated by Sonic Hedgehog during Pharyngeal Arch Development
Copyright policy )Tbx1, a DiGeorge Syndrome Candidate Gene, Is Regulated by Sonic Hedgehog during Pharyngeal Arch Development
Appropriate interactions between the epithelium and adjacent neural crest-derived mesenchyme are necessary for normal pharyngeal arch development. Disruption of pharyngeal arch development in humans underlies many of the craniofacial defects observed in the 22q11.2 deletion syndrome (del22q11), but the genes responsible remain unknown. Tbx1 is a T-box transcription factor that lies in the 22q11.2 locus. Tbx1 transcripts were found to be localized to the pharyngeal endoderm and the mesodermal core of the pharyngeal arches, but were not present in the neural crest-derived mesenchyme of the pharyngeal arches. Sonic hedgehog (Shh) is also expressed in the pharyngeal arches and is necessary for normal craniofacial development. We found that Tbx1 expression was dependent upon Shh signaling in mouse embryos, consistent with their overlapping expression in the pharyngeal arches. Furthermore, Shh was sufficient to induce Tbx1 expression when misexpressed in selected regions of chick embryos. These studies reveal a Shh-mediated pathway that regulates Tbx1 during pharyngeal arch development.
- The University of Texas Southwestern Medical Center United States
DNA, Complementary, Chromosomes, Human, Pair 22, Molecular Sequence Data, Chick Embryo, Mice, DiGeorge Syndrome, Animals, Humans, Hedgehog Proteins, Amino Acid Sequence, RNA, Messenger, Cloning, Molecular, Molecular Biology, transcription factor, In Situ Hybridization, DNA Primers, Base Sequence, Sequence Homology, Amino Acid, Sonic hedgehog, Gene Expression Regulation, Developmental, Proteins, Tbx1, Cell Biology, pharyngeal arch, 22q11.2 deletion syndrome, Pharynx, Chromosome Deletion, Developmental Biology
DNA, Complementary, Chromosomes, Human, Pair 22, Molecular Sequence Data, Chick Embryo, Mice, DiGeorge Syndrome, Animals, Humans, Hedgehog Proteins, Amino Acid Sequence, RNA, Messenger, Cloning, Molecular, Molecular Biology, transcription factor, In Situ Hybridization, DNA Primers, Base Sequence, Sequence Homology, Amino Acid, Sonic hedgehog, Gene Expression Regulation, Developmental, Proteins, Tbx1, Cell Biology, pharyngeal arch, 22q11.2 deletion syndrome, Pharynx, Chromosome Deletion, Developmental Biology
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