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Patients with hematologic and oncologic diseases are at high risk of pulmonary complications associated with abnormalities of mucociliary clearance. Respiratory infections, like RSV, are very common in pediatric patients and can be life threatening in the immunosuppressed. Additionally, about 30% of patients with sickle cell disease also have asthma and abnormal mucociliary clearance.
We have determined the crucial role that the apical cytoskeleton plays in the
development of ciliated cells of the pulmonary epithelium and shown that respiratory
diseases like RSV infection and asthma result in breakdown of the apical cytoskeleton
of ciliated cells with a consequent loss of cilia. Studies are in progress to
further identify the molecular mechanisms regulating development of differentiated
pulmonary cell types as well as to evaluate potential agents to stabilize the
apical cytoskeleton, prevent loss of cilia, and improve mucociliary clearance.
Recent Publication:
Gomperts BN, Gong-Cooper X and Hackett BP. Foxj1 regulates basal body anchoring
to the cytoskeleton of ciliated pulmonary epithelial cells. Journal of Cell
Science, 2004 Mar 15;117(Pt 8):1329-37.