Recombinant EDA or Sonic Hedgehog rescue the branching defect in Ectodysplasin A pathway mutant salivary glands in vitro.

Kirsty Wells, Chunyan Mou, Denis Headon, Abigail Tucker

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

Hypohidrotic ectodermal dysplasia (HED) is characterized by defective ectodermal organ development. This includes the salivary glands (SGs), which have an important role in lubricating the oral cavity. In humans and mice, HED is caused by mutations in Ectodysplasin A (Eda) pathway genes. Various phenotypes of the mutant mouse Eda(Ta/Ta), which lacks the ligand Eda, can be rescued by maternal injection or in vitro culture supplementation with recombinant EDA. However, the response of the SGs to this treatment has not been investigated. Here, we show that the submandibular glands (SMGs) of Eda(Ta/Ta) mice exhibit impaired branching morphogenesis, and that supplementation of Eda(Ta/Ta) SMG explants with recombinant EDA rescues the defect. Supplementation of Edar(dlJ/dlJ) SMGs with recombinant Sonic hedgehog (Shh) also rescues the defect, whereas treatment with recombinant Fgf8 does not. This work is the first to test the ability of putative Eda target molecules to rescue Eda pathway mutant SMGs.
Original languageEnglish
Pages (from-to)2674-84
Number of pages11
JournalDevelopmental Dynamics
Volume239
Issue number10
DOIs
Publication statusPublished - 2010

Keywords / Materials (for Non-textual outputs)

  • Animals
  • Ectodysplasins
  • Edar Receptor
  • Edar-Associated Death Domain Protein
  • Genotype
  • Hedgehog Proteins
  • In Situ Hybridization
  • Mice
  • Mice, Mutant Strains
  • Morphogenesis
  • Organ Culture Techniques
  • Salivary Glands
  • Signal Transduction

Fingerprint

Dive into the research topics of 'Recombinant EDA or Sonic Hedgehog rescue the branching defect in Ectodysplasin A pathway mutant salivary glands in vitro.'. Together they form a unique fingerprint.

Cite this