Tbx1 and Brn4 regulate retinoic acid metabolic genes during cochlear morphogenesis.

ABSTRACT:

Background

In vertebrates, the inner ear is comprised of the cochlea and vestibular system, which develop from the otic vesicle. This process is regulated via inductive interactions from surrounding tissues. Tbx1, the gene responsible for velo-cardio-facial syndrome/DiGeorge syndrome in humans, is required for ear development in mice. Tbx1 is expressed in the otic epithelium and adjacent periotic mesenchyme (POM), and both of these domains are required for inner ear formation. To study the function of Tbx1 in the POM, we have conditionally inactivated Tbx1 in the mesoderm while keeping expression in the otic vesicle intact.

Results

Conditional mutants (TCre-KO) displayed malformed inner ears, including a hypoplastic otic vesicle and a severely shortened cochlear duct, indica

SUBMITTER: Braunstein EM 

PROVIDER: S-EPMC2700094 | biostudies-literature | 2009 May

REPOSITORIES: biostudies-literature