DEDICATED TO IMPROVING THE LIVES OF Those AFFECTED BY CYSTIC FIBROSIS
Student CF Research Award Recipients: Summer 2017
Franny Kiles is a sophomore biochemistry major at Tufts University. She performed a project related to personalized medicine for rare mutations in the CF gene. Franny tested CFTR modulator responses and learned to use the electrophysiological CFTR function assay. She studied nasal epithelial cell cultures from CF patients with one F508del mutation and one ultra rare CFTR mutation e.g. S1159P or c.850dupA. Franny’s work is aimed at making sure that no CF patient is overlooked to receive CFTR-based drug treatment due to the rarity of their mutations.
Gopika Hari is a junior at Virginia Commonwealth University. This is her second summer student award. In collaboration with scientists at CHORI and Stanford University, Gopika examined the physiological role of Prostaglandin-E2, a hormone-like lipid compound that is produced during inflammation. Her work described the defective response to prostaglandin-E2 in terms of chloride and bicarbonate secretion in CF airway epithelial cells. Her findings identified a new factor that contributes to CF airway disease. Results from Gopika’s work have been recently published in a peer-reviewed article (Sellers, Zachary M., Beate Illek, Miriam Frankenthal Figueira, Gopika Hari, Nam Soo Joo, Eric Sibley, Jackson Souza-Menezes, Marcelo M. Morales, Horst Fischer, and Jeffrey J. Wine. "Impaired PGE2-stimulated Cl-and HCO3-secretion contributes to CF airway disease." PloS one 12, no. 12 (2017): e0189894).
Student CF Research Award Publications
Illek B, Zlock L, Boecking C, Green B, Lefebvre E, Bates M, McGarry M, Nielson D, Finkbeiner W (2016) CFTR function in CF Patient-derived airway epithelial cells with common and rare mutations. Ped. Pulmonol. 51, suppl. 40, 198-199.
Sellers ZM, Illek B, Figueria M, Hari G, Joo NS, Sibley E, Fischer H, Wine JJ (2015) PGE2-stimulated Cl- secretion and mucociliary clearance in cystic fibrosis airway. Ped. Pulmonol. 50, suppl. 39, 236.
Fischer H, Echavia K, Finkbeiner W, Illek B (2015). Growth inhibition of P. aeruginosa by an alkaline airway pH in a bacterial-epithelial coculture system. Ped. Pulmonol. 50, suppl. 39, 238.
Illek B, Zlock L, Boecking C, Olshansky S, Echavia K, Gopika H, Pangilinan E, Finkbeiner WE, Fischer H (2015) Conditionally reprogrammed cells for personalized medicine in cystic fibrosis. Ped. Pulmonol. 50, suppl 39, 256-257.
Illek B, Meiss L, Modlin S, Cohen F, Gruenert DC, Fischer H (2010). Hyperactivation and protection of CFTR Cl transport by brief hyperthermic treatment. Ped. Pulmonol. 45, suppl 33, 257.
Illek B, Bustillo ME; Spence, S, Fischer H, Gruenert D (2011) Enhancement of CFTR correction by brief hyperthermic treatment and DNA methyltransferase inhibitor. Ped. Pulmonol. 46, suppl 34, 232.
Lauren Meiss, Role of CFTR and p53 during Pseudomonas Aeruginosa Homoserine Lactone-Induced Apoptosis and Epithelial Barrier Breakdown. Honor Thesis. Arizona State University.
lllek B, Meiss L, Modlin S, Cohen F, Gruenert D, Howard M, Fischer H (2009) Heat shock response protects against pyocyanin-induced loss of CFTR function in bronchial epithelial cells. Free Rad Biol Med., 47 suppl 1, S101.
lllek B, Fischer H, Streich-Tilles T, Cheng A, Ames BN, Jiang Q, Peden DB (2007) Duox1-mediated release of hydrogen peroxide is modified by vitamin C. Am J Respir. Crit. Care Med., A126.
Fischer H, Streich-Tilles T, Illek B (2008) Expression and Function of the HVCN1 proton channel in airways. (2008). Ped. Pulmonol., suppl 31, 244.
Illek, B, Suh, JH, Kim E, Barber KJ, Fischer H, Gruenert D, Schwarzer C, Machen TE (2008) Oxidative stress by pyocyanin inhibits Cl- transport in wildtype CFTR corrected cystic fibrosis bronchial epithelial cells. Ped. Pulmonol., suppl 31,228, 322.
Schwarzer C , Fischer H, Kim EJ, Barber KJ, Mills AD, Kurth MJ, Gruenert DC, Suh JH, Machen TE, Illek B (2008) Oxidative Stress caused by pyocyanin impairs CFTR Cl- Transport In Human Bronchial Epithelial Cells. Free Radical Biology & Medicine. 45(12): 1653-62.
Maurisse R, Illek B, Cheung J, Wahler L, Fischer H, Gruenert DC (2006) Episomal expression of the entire 6.2kb CFTR cDNA corrects defective Cl secretion across polarized cystic fibrosis airways. Ped. Pulmonol., suppl. 29, 290.
Illek B, Maurisse R, Fischer H, Wahler L, Gruenert DC (2007). Enhanced ATP-stimulated Cl secretion after complementation of CF airway epithelia cells with WT-CFTR. Ped. Pulmonol., suppl.30, 250.
Illek B, Maurisse R, Wahler L, Kunzelmann K, Fischer H, Gruenert DC (2008) Cl- Transport in Complemented CF Bronchial Epithelial Cells Correlates with CFTR mRNA Expression Levels. J. Cellular Physiol. Biochem. 22:57-68.