Student CF Research Award Recipient: Summer 2018

​Franny Kiles is a junior majoring in biology at Tufts University in Boston, MA. This was her 2nd ENF Student Research Award. Her project looked at the mechanism of Prostaglandin E2 (PGE2) in stimulating chloride secretion in human bronchial epithelial cells. Franny    studied how much PGE2 intracellular signaling involved CFTR versus other chloride ion channels. The work determined that PGE2 mainly acts on a specific receptor, which leads to activation of protein kinase A and calcium cross talk signaling pathways that ultimately involve CFTR. Through its stimulation of chloride secretion, PGE2 plays an important role in mucus clearance. Therefore, a better understanding of the PGE2 signaling pathway could lead to potential treatments for CF. The results of her work have been submitted for publication. 

Student CF Research Award Publications

Abazari S, Hari G, Crowther E,  Kiles F, Trumbull A, Illek B, Sellers ZM (2019) Prostaglandin E2-Stimulated Cl-Secretion
in Airway Epithelium Involves Protein Kinase A and Calcium Crosstalk. FASEB J (submitted). 
 
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.
 
Bustillo ME, Juarez L, Meiss L, Fischer H, Gruenert DC, Illek B (2013). Heat Shock Activates Transgene CFTR Cl Channels. FASEB J, Apr 2013; 26:696.1
 
Illek B, Fischer HB, Bustillo ME, Meiss L, Juarez L, Gruenert  DC, (2013) . Heat Shock Enhances CMV      promoter-driven CFTR Expression in CF airways. Ped. Pulmonol. 48, suppl. 35, 230.  
  
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.

"I was diagnosed with CF at 2 months of age. I have spent my whole life thinking about CF from a patient’s point of view, but this internship allowed me to think about CF from a scientific perspective as well. It has been such an amazing experience conducting CF research with Dr. Illek for the past two summers."            Franny