CONGENITAL DIAPHRAGMATIC HERNIA (CDH)
Our proposed research has been designed to determine the normal developmental
processes underlying phrenic nerve-diaphragm formation and then to establish
which aspects maldevelop in a well-established rodent model of CDH. CDH is a
developmental anomaly characterized by large regions of the diaphragm failing
to form. Consequently, the developing viscera invade the thoracic cavity,
occupying space normally reserved to accommodate the growing lung. As a result,
newborns with CDH (~1:3000 births; ~50% mortality) suffer from a combination of
pulmonary hypoplasia, pulmonary hypertension and surfactant deficiency.
Methods: Immunohistochemistry; in situ hybridization; whole embryo cultures; use
of null-mutant mice; examining retinoid signaling pathways in cells lines and
transgenic mice.
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