Sonic hedgehog is essential to foregut development.

Litingtung Y, Lei L, Westphal H, Chiang C
Nat Genet. 1998 20 (1): 58-61

PMID: 9731532 · DOI:10.1038/1717

Congenital malformation of the foregut is common in humans, with an estimated incidence of 1 in 3000 live births, although its aetiology remains largely unknown. Mice with a targeted deletion of Sonic hedgehog (Shh) have foregut defects that are apparent as early as embryonic day 9.5, when the tracheal diverticulum begins to outgrow. Homozygous Shh-null mutant mice show oesophageal atresia/stenosis, tracheo-oesophageal fistula and tracheal and lung anomalies, features similar to those observed in humans with foregut defects. The lung mesenchyme shows enhanced cell death, decreased cell proliferation and downregulation of Shh target genes. These results indicate that Shh is required for the growth and differentiation of the oesophagus, trachea and lung, and suggest that mutations in SHH and its signalling components may be involved in foregut defects in humans.

MeSH Terms (26)

Animals DNA-Binding Proteins Endoderm Esophagus Gene Expression Regulation, Developmental Hedgehog Proteins Immunohistochemistry In Situ Hybridization Kruppel-Like Transcription Factors Lung Membrane Proteins Mesoderm Mice Mice, Knockout Nerve Tissue Proteins Organ Culture Techniques Patched Receptors Proteins Receptors, Cell Surface Repressor Proteins Time Factors Trachea Trans-Activators Transcription Factors Xenopus Proteins Zinc Finger Protein Gli3

Connections (1)

This publication is referenced by other Labnodes entities: