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Recombinant EDA or Sonic Hedgehog rescue the branching defect in Ectodysplasin A pathway mutant salivary glands in vitro
Author(s) -
Wells K.L.,
Mou C.,
Headon D.J.,
Tucker A.S.
Publication year - 2010
Publication title -
developmental dynamics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.634
H-Index - 141
eISSN - 1097-0177
pISSN - 1058-8388
DOI - 10.1002/dvdy.22406
Subject(s) - hypohidrotic ectodermal dysplasia , biology , recombinant dna , sonic hedgehog , ectodermal dysplasia , mutant , hedgehog signaling pathway , microbiology and biotechnology , phenotype , genetics , signal transduction , gene
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. Developmental Dynamics 239:2674–2684, 2010. © 2010 Wiley‐Liss, Inc.