Reduced Vipergic Innervation In Duodenum Tissue of cf Mice

Cystic Fibrosis (CF) is the most common fatal genetic disease in white populations. It is caused by mutations in the CFTR gene that cause the dysfunction or absence of the protein CFTR (Cystic Fibrosis Transmembrane conductance Regulator) from the respiratory and digestive system. CFTR dysfunction causes severe damage to the lungs, pancreas and intestines due to the buildup of thick and viscous mucus leading to malabsorption of nutrients and severe obstructive lung disease with chronic inflammation and bacterial infection. Respiratory failure is the major cause of death for CF patients. Despite much progress in the treatment of CF that has improved patient's prognosis and quality of life, treating the molecular root of the disease is still a major challenge and not available to most CF patients. Previous studies from the Chappe lab indicate that the Vasoactive Intestinal Peptide (VIP), an important natural peptide (a form of a very tiny protein) of the body that controls CFTR to maintain healthy lungs and digestive system, could play a major role in CF disease development and progression. Unfortunately, VIP which is normally released by nerves surrounding the lungs, intestine and pancreas, appears to be missing or poorly present in CF. In the present study, I have measured the amount of VIP in the lung, sweat glands, intestine, pancreas and salivary glands of 8- to 17-week-old CF mice compared to same age healthy tissues. I am also investigating changes in nerves that produce VIP. Since VIP is a key peptide for the function of the CFTR protein, this research will result in new knowledge of an important under-studied aspect of the disease and will provide some cues for the development of a VIP-based therapy to treat CF.