Examples of Recent CF-Related Publications by UAB Investigators (2006-2011)

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz J, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein R, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordonez CL, Campbell PW, Ashlock MA, Ramsey BW. Effect of VX-770 in persons with cystic fibrosis and the G551D CFTR mutation. New England Journal of Medicine, 363(21):1991-2003, 2010.

Alexander NS, Hatch N, Zhang S, Skinner D, Fortenberry J, Sorscher EJ, Woodworth BA. Resveratrol has salutary effects on mucociliary transport and inflammation in sinonasal epithelium. Laryngoscope 121(6):1313-9, 2011.

Atwell S, Brouillette CG, Conners K, Emtage S, Gheyi T, Guggino WB, Hendle J, Hunt JF, Lewis HA, Lu F, Protasevich II, Rodgers LA, Romero R, Wasserman SR, Weber PC, Wetmore D, Zhang FF, Zhao X. Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant. Protein Engineering, Design and Selection, 23(5):375-84, 2010.

Azbell C, Zhang S, Skinner D, Fortenberry J, Sorscher EJ, Woodworth BA. Hesperidin stimulates cystic fibrosis transmembrane conductance regulator-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium. Otolaryngol Head Neck Surgery, 143(3): 397-404, 2010.

Bartoszewski R, Rab A, Fu L, Bartoszewska S, Collawn J, Bebok Z. CFTR expression regulation by the unfolded protein response. Methods in Enzymology, 491:3-24, 2011.

Bartoszewski R, Rab A, Jurkuvenaite A, Mazur M, Wakefield J, Collawn JF, Bebok Z. Activation of the unfolded protein response by DeltaF508 CFTR. American Journal of Respiratory Cell & Molecular Biology. 39: 448-57, 2008.

Bartoszewski R, Rab A, Twitty G, Stevenson L, Fortenberry J, Piotrowski A, Dumanski JP, Bebok Z. The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response. Journal of Biological Chemistry. 83(18):12154-65, 2008.

Bartoszewski, R, Jablonsky M, Bartoszewski, S, Stevenson L, Dai Q, Kappes J, Collawn JF, Bebok Z. A synonymous single nucleotide polymorphism in ΔF508 CFTR alters the secondary structure of the mRNA and expression of the mutant protein. Journal of Biological Chemistry. 285(37): 28741-8, 2010.

Bates E, Miller S, Alexander M, Mazur M, Fortenberry JA, Bebok Z, Sorscher EJ, Rowe SM. Bioelectric effects of quinine on polarized airway epithelial cells. Journal of Cystic Fibrosis. 6(5):351-9, 2007.

Berdiev BK, Cormet-Boyaka E, Tousson A, Qadri YJ, Oosterveld-Hut HM, Hong JS, Gonzales PA, Fuller CM, Sorscher EJ, Lukacs GL, Benos DJ. Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer. Journal of Biological Chemistry. 82(50): 36481-8, 2007.

Berdiev BK, Qadri YJ, Benos DJ. Assessment of the CFTR and ENaC association. Molecular Biosystems. 5(2):123-7, 2009.

Bernard K, Wang W, Narlawar R, Schmidt B, Kirk KL. Curcumin cross-links cystic fibrosis transmembrane conductance regulator (CFTR) polypeptides and potentiates CFTR channel activity by distinct mechanisms. Journal of Biological Chemistry. 84(45):30754-65. 2009.

Bernard K, Kirk KL. Cross-linking of ΔF508-CFTR promotes its trafficking to the plasma membrane. Channels (Austin): 4(4):251-4, 2010.

Blount A, Zhang S, Chestnut M, Hixon B, Skinner D, Sorscher EJ, Woodword BA.  Transepithelial ion transport is suppressed in hypoxic sinonasal epithelium.  Laryngoscope 121(9):1929-34, 2011.

Britton LJ, Saeed SA. Abdominal pain in cystic fibrosis. Journal of Pediatric Health Care. 22:383-6, 2008.

Britton LJ, Thrasher S, Gutierrez H. Creating a culture of improvement: experience of a pediatric cystic fibrosis center. Journal of Nursing Care Quality. 23:115-20; 121-2, 2008.

Chen L, Bosworth CA, Pico T, Collawn JF, Varga K, Gao Z, Clancy JP, Fortenberry JA, Lancaster JR Jr., Matalon S. DETANO and nitrated lipids increase chloride secretion across lung airway cells. American Journal of Respiratory Cell & Molecular Biology. 39(2): 150-62, 2008.

Chen L, Patel RP, Teng X, Bosworth CA, Lancaster JR Jr., Matalon S. Mechanisms of cystic fibrosis transmembrane conductance regulator activation by S-nitrosoglutathione. Journal of Biological Chemistry. 281(14):9190-9, 2006.

Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, Jain M, Leonard A, McCoy KS, Moss RB, Pilewski JM, Rosenbluth DB, Rubenstein RC, Schechter MS, Botfield M, Ordoñez CL, Spencer-Green GT, Vernillet L, Wisseh S, Yen K, Konstan MW. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax, Nov. 5, 2011. [Epub ahead of print]

Clancy JP, Rowe SM, Bebok Z, Aitken ML, Gibson R, Zeitlin P, Berclaz P, Moss R, Knowles MR, Oster RA, Mayer-Hamblett N, Ramsey B. No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. American Journal of Respiratory Cell & Molecular Biology. 37(1):57-66, 2007.

Clancy, JP. Emerging therapies for cystic fibrosis lung disease (review). US Respiratory Disease. In press, 2011.

Clancy, JP. Diagnosing cystic fibrosis in patients with non-diagnostic results: the case for intestinal current measurements. Thorax. (7):575-6, 2010.

Collawn JF, Bebok Z, Matalon S. Search and rescue: finding ways to correct deltaF508 CFTR. American Journal of Respiratory Cell & Molecular Biology. 40(4): 385-7, 2009.

Collawn JF, Fu L, Bebok Z. Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator. Expert Review of Proteomics. 7(4):495-506, 2010.

Com G, Clancy JP. Adenosine receptors, cystic fibrosis, and airway hydration. Handbook of Experimental Pharmacology. 193:363-81, 2009.

Cormet-Boyaka E, Hong JS, Berdiev BK, Fortenberry J, Rennolds J, Clancy JP, Benos DJ, Boyaka PN, Sorscher EJ. A truncated CFTR gene rescues endogenous ∆F508-CFTR and corrects chloride transport in CF mice. The FASEB Journal. 23(11):3743-51, 2009.

Du M, Keeling KM, Fan L, Liu X, Bedwell DM. Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model. Journal of Biological Chemistry. 13;284(11): 6885-92, 2009.

Du M, Keeling KM, Fan L, Liu X, Kovacs T, Sorscher EJ, Bedwell DM. Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model. Journal of Molecular Medicine. 84(7):573-82, 2006.

Du M, Liu X, Welch EM, Hirawat S, Peltz SW, Bedwell DM. PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model. Proceedings of the National Academy of Sciences USA. 105(6): 2064-9, 2008.

Dulong S, Bernard K, Ehrenfeld J. Enhancement of P2Y6-induced Cl- secretion by IL-13 and modulation of SK4 channels activity in human bronchial cells. Cellular Physiology and Biochemistry. 20(5):483-94, 2007.

Ehrhardt A, Pyle LC, Wang W, Nowotarski K, Mulvihill C, di Bernardo S, Ramjeesingh M, Lewis HA, Atwell S, Bear CE, Lukacs GL, Kirk KL, Sorscher EJ. Biochemical and functional interactions between the nucleotide binding domains and cytosolic loops of CFTR.  In Revision, 2011.

Falany CN, He D, Li L, Falany JL, Wilborn TW, Kocarek TA, Runge-Morris M. Regulation of hepatic sulfotransferase (SULT) 1E1 expression and effects on estrogenic activity in cystic fibrosis (CF). The Journal of Steroid Biochemistry and Molecular Biology. 114 (1-2):113-119, 2009.

Flume et al. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. AJP-Crit Care Med. 182: 298-306, 2010.

Fu L, Rab A, Tang L, Rowe SM, Bebok Z, Collawn JF. Dab2 is a Key Regulator of Endocytosis and Post-endocytic Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator. Biochem J. Oct. 13, 2011. [Epub ahead of print]

Fu L, Sztul E. ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy. European Journal of Cell Biology. 88(4):215-26, 2009.

Gaggar A, Hector A, Bratcher PE, Mall MA, Griese M, Hartl D. The role of

matrix metalloproteinases in cystic fibrosis lung disease. European Respiratory Journal. 38(3):721-7, 2011.

Gaggar A, Jackson PL, Noerager BD, O'Reilly PJ, McQuaid DB, Rowe SM, Clancy JP, Blalock JE. A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. Journal of Immunology. 180(8):5662-9, 2008.

Gaggar A, Li Y, Weathington N, Winkler M, Kong M, Jackson P, Blalock JE, Clancy JP. Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients. American Journal of Physiology Lung Cellular and Molecular Physiology. 293(1):L96-L104, 2007.

Gaggar A, Rowe SM, Matthew H, Blalock JE. Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation. The Open Respiratory Medicine Journal. 4:32-8, 2010.

Ganeshan R, Nowotarski K, Di A, Nelson DJ, Kirk KL. CFTR surface expression and chloride currents are decreased by inhibitors of N-WASP and actin polymerization. Biochimica et Biophysica Acta. 1773(2):192-200, 2007.

Goldstein RF, Niraj A, Sanderson TP, Wilson LS, Rab A, Kim H, Bebok Z, Collawn JF. VCP/p97 AAA-ATPase does not interact with the endogenous wild-type cystic fibrosis transmembrane conductance regulator. American Journal of Respiratory Cell & Molecular Biology. 36(6):706-14, 2007.

Griese M, Kappler M, Gaggar A, Hartl D. Inhibition of airway proteases in cystic fibrosis lung disease. European Respiratory Journal. 32(3):783-95, 2008.

Guimbellot J, Erickson SW, Wen H, Page GP, Sorscher EJ, Hong JS. Correlation of microRNA levels in CFTR during hypoxia with predicted target mRNAs through genome-wide microarray analysis. BMC Medical Genomics. 2:15, 2009.

Guimbellot JS, Fortenberry JA, Siegal GP, Moore B, Wen H, Venglarik C, Chen, YF, Oparil S, Sorscher EJ, Hong JS. Role of oxygen availability in cystic fibrosis transmembrane conductance regulator expression and function. American Journal of Respiratory Cell & Molecular Biology. 39: 514-21, 2008.

Gutierrez HH, Sanchez I, Schidlow DV. Cystic fibrosis care in Chile. Current Opinion in Pulmonary Medicine. 15(6): 632-637, 2009.

Hallows KR, Fitch AC, Richardson CA, Reynolds PR, Clancy JP, Dagher P, Witters LA, Kolls JK, Pilewski JM. Up-regulation of AMP-activated kinase by dysfunctional cystic fibrosis transmembrane conductance regulator in cystic fibrosis airway epithelial cells mitigates excessive inflammation. Journal of Biological Chemistry. 281(7):4231-41, 2006.

Hardison MT, Galin FS, Calderon CE, Djekic UV, Parker SB, Wille KM, Jackson PL, Oster RA, Young KR, Blalock JE, Gaggar A. The presence of a matrix-derived neutrophil chemoattractant in bronchiolitis obliterans syndrome after lung transplantation. (Note: includes a cohort of CF individuals post lung transplantation) Journal of Immunology. 1;182(7):4423-31, 2009.

Harris WT, Muhlebach MS, Oster RA, Knowles MR, Noah TL. Transforming growth factor-beta(1) in bronchoalveolar lavage fluid from children with cystic fibrosis. Pediatric Pulmonology. 44(11):1057-64, 2009.

Harris WT, Muhlebach MS, Oster RA, Knowles MR, Clancy JP, Noah TL. Plasma TGF-β₁ in pediatric cystic fibrosis: potential biomarker of lung disease and response to therapy. Pediatric Pulmonology. 46(7):688-95, 2011.

Havasi V, Keiles S, Hambuch T, Sorscher EJ, Kammesheidt A. The role of the F508C mutation in congenital bilateral absence of the vas deferens. Genetics in Medicine. 10(12):910-4, 2008.

Havasi V, Rowe SM, Kolettis PN, Dayangac D, Sahin A, Grangeia A, Carvalho F, Barros A, Sousa M, Bassas L, Casals T, Sorscher EJ. Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens. Journal of Fertility and Sterility. 94(6):2122-7, 2010.

Havasi V, Sorscher EJ, Hurst C, Briles T, Yang F, Bains D. Inhibitory effects of hypertonic saline on P. aeruginosa motility. Journal of Cystic Fibrosis. 7(4):267-9, 2008.

He D, Wilborn TW, Falany JL. Li L and Falany CN. Repression of CFTR activity in human MMNK-1  cholangiocytes induces sulfotransferase 1E1 expression in co-cultured HepG2 hepatocytes. Biochimica et  Biophysica Acta 1783(12), 2391-2397, 2008.

Hill AE, Hong JS, Wen H, Teng L, McPherson DT, McPherson SA, Levasseur DN, Sorscher EJ. Micro-RNA-like effects of complete intronic sequences in CFTR. Frontiers in Bioscience. 11:1998-2006, 2006.

Horvath G, Sorscher EJ. Luminal fluid tonicity regulates airway ciliary beating by altering membrane stretch and intracellular calcium. Cell Motility and the Cytoskeleton. 65(6): 469-75, 2008.

Houser GH, Holt CL, Clancy JP, Leon K, Rowe SM, Gaggar A, Gutierrez HH, Young KR, Robin NH. Genetic and reproductive knowledge among adolescents and adults with cystic fibrosis. Chest 133:1533, 2008.

Hutt DM, Herman D, Rodrigues AP, Noel S, Pilewski JM, Matteson J, Hoch B, Kellner W, Kelly JW, Schmidt A, Thomas PJ, Matsumura Y, Skach WR, Gentzsch M, Riordan JR, Sorscher EJ, Okyoneda T, Yates JR, Lukas GL, Frizell RA, Manning G, Gottesfeld JM, Balch WE. Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nature Chemical Biology. 6(1):25-33, 2010.

Jackson PL, Noerager BD, Jablonsky MJ, Hardison MT, Cox BD, Patterson JC, Dhanapal B, Blalock JE, Muccio DD. A CXCL8 receptor antagonist based on the structure of N-acetyl-proline-glycine-proline. European Journal of Pharmacology. 668(3):435-42, 2011.

Jackson PL, Xu X, Wilson L, Weathington NM, Clancy JP, Blalock JE, Gaggar A. Human neutrophil elastase-mediated cleavage sites of MMP-9 and TIMP-1: Implications to cystic fibrosis proteolytic dysfunction. Molecular Medicine. 16 (5-6) 159-166, 2010.

Ji HL, Su XF, Li J, Barbry P, Smith PR, Matalon S, Benos, DJ. δ-Subunit confers novel biophysical features to αβγ-human epithelial sodium channel (ENaC) via a physical interaction. Journal of Biological Chemistry; 281:8233-41 2006.

Jurkuvenaite A, Chen L, Bartoszewski R, Goldstein R, Bebok Z, Matalon S, Collawn JF. Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) in airway epithelial cells. American Journal of Respiratory Cell & Molecular Biology. 42(3): 363-72, 2010.

Jurkuvenaite A, Varga K, Nowotarski K, Kirk KL, Sorscher EJ, Li Y, Clancy JP, Bebok Z, Collawn JF. Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis. Journal of Biological Chemistry. 281(6):3329-34, 2006.

Khazai NB, Judd  SE, Jen L,  Wolfenden LL, Stecenko A, Ziegler TR, Tangpricha V. Treatment and prevention of vitamin D insufficiency in cystic fibrosis patients: comparative efficacy of ergocalciferol, cholecalciferol, and UV light. Journal of Clinical Endocrinology and Metabolism 94(6): 2037-43, 2009.

Kirk KL. CFTR channels and wound healing. Focus on "Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair. American Journal of Physiology, Cell Physiology. 299(5):C888-90, 2010.

Kirk K, Wang W. A unified view of CFTR gating: combining the allosterism of a ligand-gated channel with the enzymatic activity of an ABC transporter. Journal of Biological Chemistry 286(15):12813-9, 2011.

Koller B, Kappler M, Latzin P, Gaggar A, Schreiner M, Takyar S, Kormann M, Kabesch M, Roos D, Griese M, Hartl D. TLR expression on neutrophils at the pulmonary site of infection: TLR1/ TLR2-mediated up-regulation of TLR5 expression in cystic fibrosis lung disease. Journal of Immunology. 181:2753-63, 2008.

Kreindler JL, Steele C, Nguyen N, Chan YR, Pilewski JM, Alcorn JF, Vyas YM, Aujla SJ, Finelli P, Blanchard M, Zeigler SF, Logar A, Hartigan E, Kurs-Lasky M, Rockette H, Ray A, Kolls JK. Vitamin D3 attenuates Th2 responses to Aspergillus fumigatus mounted by CD4+ T cells from cystic fibrosis patients with allergic bronchopulmonary aspergillosis. J Clinical Investigation. 120:3242, 2010.

Lazrak A, Jurkuvenaite A, Chen L, Keeling KM, Collawn JF, Bedwell DM, Matalon  S. Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung. American Journal of Physiology - Lung Cellular and Molecular Physiology. 301(4):L557-67, 2011.

Li L, He D, Wilborn TW, Falany JL and Falany CN. Increased SULT1E1 activity in HepG2 hepatocytes decreases growth hormone stimulation of STAT5b phosphorylation. Steroids 74: 20-29, 2009.

Li L, Falany CN. Elevated hepatic SULT1E1 activity in mouse models of cystic fibrosis alters the regulation of estrogen responsive proteins. Journal of Cystic Fibrosis. 6(1):23-30, 2007.

Li Y, Wang W, Parker W, Clancy JP. Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia. American Journal of Respiratory Cell & Molecular Biology. 34(5): 600-8, 2006.

McClure M, Delucas LJ, Wilson L, Ray M, Rowe SM, Wu X, Dai Q, Hong JS, Sorscher EJ, Kappes JC, Barnes S. Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications. Protein Engineering Design and Selection. 2011 [Epub ahead of print]

Meltzer RH, Kapoor N, Yawar YJ, Anderson SJ, Fuller CM, Benos DJ. Heteromeric Assembly of acid-sensitive ion channel and epithelial sodium channel subunits. Journal of Biological Chemistry, 282, 25548-25559, 2007.

Moss RB, Milla C, Colombo J, Accurso F, Zeitlin PL, Clancy JP, Spencer LT, Pilewski J, Waltz DA, Dorkin HL, Ferkol T, Pian M, Ramsey B, Carter BJ, Martin DB, Heald AE. Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial     . Human Gene Therapy. 18(8):726-32, 2007.

Nie HG, Chen L, Han DY, Li J, Song WF, Wei SP, Fang XH, Gu X, Matalon S, Ji HL. Regulation of epithelial sodium channels by cGMP/PKGII. The Journal of Physiology. 587(Pt 11):2663-76, 2009.

Noah TL, Ivins SS, Abode KA, Stewart PW, Michelson PH, Harris WT, Henry MM, Leigh MW. Inhaled versus systemic antibiotics and airway inflammation in children with cystic fibrosis and Pseudomonas. Pediatric Pulmonology. 45(3):281-90, 2010.

Peters KW, Okiyoneda T, Balch WE, Braakman I, Brodsky JL, Guggino WB, Penland CM, Pollard HB, Sorscher EJ, Skach WR, Thomas PJ, Lukacs GL, Frizzell RA.  CFTR Folding Consortium:  methods available for studies of CFTR folding and correction.  Methods in Molecular Biology. 742:335-53, 2011. (PMCID: PMC32-5413)

Peterson-Carmichael SL, Harris WT, Goel R, Noah TL, Johnson R, Leigh MW, Davis SD. Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis. Pediatric Pulmonology. 44(5):503-11, 2009.

Protasevich I, Yang Z, Wang C, Atwell S, Zhao X, Emtage S, Wetmore D, Hunt JF, Brouillette CG. Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1. Protein Science. 19(10):1917-31, 2010.

Pyle LC, Ehrhardt A, Mitchell LH, Fan L, Ren A, Naren AP, Li Y, Clancy JP, Bolger GB, Sorscher EJ, Rowe SM. Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators. American Journal of Physiology - Lung Cellular and Molecular Physiology. 301(4):L587-97, 2011.

Pyle LC, Fulton JC, Sloane PA, Backer K, Mazur M, Prasain J, Barnes S, Clancy JP, Rowe SM. Activation of CFTR by the flavonoid quercetin: Potential use as a biomarker of delta F508 CFTR rescue. American Journal of Respiratory Cell and Molecular Biology. 43(5):607-16, 2010.

Rab A, Bartoszewski R, Jurkuvenaite A, Wakefield J, Collawn JF, Bebok Z. Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression. American Journal of Physiology Cell Physiology. 292(2):C756-66, 2007.

Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, DÅ™evínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. New England Journal of Medicine. 365(18):1663-72, 2011.

Rennolds J, Tower C, Musgrove L, Fan L, Maloney K, Clancy JP, Kirk KL, Sztul E, Cormet-Boyaka E. Cystic fibrosis transmembrane conductance regulator trafficking is mediated by the COPI coat in epithelial cells. Journal of Biological Chemistry. 283(2): 833-9, 2008.

Rosenfeld M, Emerson J, McNamara S, Joubran K, Retsch-Bogart G, Graff GR, Gutierrez HH, Kanga JF, Lahiri T, Noyes B, Ramsey B, Ren CL, Schechter M, Morgan W, Gibson RL; EPIC Study Group Participating Clinical Sites. Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort. Pediatric Pulmonology 45(9): 934-44, 2010. 

Rowe SM, Accurso F, Clancy JP. Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proceedings of the American Thoracic Society. 4(4):387-98, 2007.

Rowe SM, Clancy JP. Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development. BioDrugs. 23(3):165-74, 2009.

Rowe SM, Clancy JP. Advances in cystic fibrosis therapies. Current Opinion in Pediatrics. 18(6):604-613, 2006.

Rowe SM, Clancy JP, Sorscher EJ. A Breath of Fresh Air. Scientific American, 305(2):68-73, 2011.

Rowe SM, Clancy JP, Wilschanski M. Nasal potential difference measurements to  assess CFTR ion channel activity. Methods in Molecular Biology. 741:69-86, 2011.

Rowe SM, Jackson PL, Liu G, Hardison M, Livraghi A, Solomon GM, McQuaid DB, Noerager BD, Gaggar A, Clancy JP, O'Neal W, Sorscher EJ, Abraham E, Blalock JE. Potential role of high mobility group box 1 in cystic fibrosis pulmonary disease. American Journal of Respiratory Critical Care Medicine. 178:822-31, 2008.

Rowe SM, Varga K, Rab A, Bebok Z, Byram K, Li Y, Sorscher EJ, Clancy JP. Restoration of W1282X CFTR activity by enhanced expression. American Journal of Respiratory Cell & Molecular Biology. 37(3): 347-56, 2007.

Rowe SM, Pyle LC, Jurkuvenaite A, Varga K, Collawn J, Sloane PA, Woodworth B, Mazur M, Fulton J, Fan L, Li Y, Fortenberry J, Sorscher EJ, Clancy JP. ∆F508 CFTR correction and activity in polarized airway and non-airway cell monolayers. Pulmonary Pharmacology and Therapeutics. 23(4): 268-78, 2010.

Rowe SM, Sloane P, Tang LP, Backer K, Mazur M, Buckley-Lanier J, Nudelman I, Belakhov V, Bebok Z, Schwiebert E, Baasov T, Bedwell DM. Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54. J Molecular Medicine (Berl). 89(11):1149-61, 2011.

Schechter, M. S. and H. H. Gutierrez. Improving the quality of care for patients with cystic fibrosis. Current Opinion in Pediatrics 22(3): 296-301, 2010.

Schwiebert EM. Extracellular autocrine nucleotide signaling in a microenvironment: integrative physiology in a minute volume of airway surface liquid. Journal of Physiology. 580(Pt. 2):359-60, 2007.

Seifert CM, Harvey RJ, Mathews JW, Meyer TA, Ahn C, Woodworth VA, Schlosser RJ. Temporal bone pneumatization and its relationship to paranasal sinus development in cystic fibrosis. Rhinology 48(2):233-238, 2010.

Sloane PA, Rowe SM. Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis. Current Opinion in Pulmonary Medicine 16(6);591-7, 2010.

Snelgrove RJ, Jackson PL, Noerager BD, Hardison MT, Kinloch A,Gaggar A, Shastry S, Rowe SM, Shim YM, Hussell T, Blalock JE. A critical role for LTA4H in limiting chronic pulmonary inflammation. Science, 330(6000):40-1, 2010.

Solomon GM, Konstan MW, Wilschanski, M, Billings J, Sermet I, Accurso FJ, Vermeleun LE, Hathorne H, Reeves G, Sabbatini G, Hill AE, Mayer-Hamblett N, Ashlock M, Clancy JP, Rowe SM. An international randomized multicenter comparison of nasal potential difference techniques. Chest 138(4):919-28, 2010.

Sorscher EJ, Harris J, Alexander M, Rottgers A, Hardy K, Ponnazhagan S, Collawn JF, McClintock J, Amsler CD, Webster A, Maddry J, Baker BJ, Hong JS. Activators of viral gene expression in polarized epithelial monolayers identified by rapid-throughput drug screening. Gene Therapy. 13(9):781-8, 2006.

Stalvey MS, Anbar RD, Konstan MW, Jacobs JR, Bakker B, Lippe B, Geller DE. A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatric Pulmonology. Sep. 8, 2011 [Epub ahead of print]

Su X-F, Cormet-Boyaka E, Smith PR, Kirk KL, Sorscher EJ, Benos DJ, Matalon S, Ji HL. Proton-gated Na+ channel (ASIC3) interacts with cystic fibrosis transmembrane conductance regulator in Calu-3 cells. Journal of Biological Chemistry 281:36960-8, 2006.

Troxler, RB, Hoover WC, Britton LJ, Gerwin AM, Rowe SM. Eradication of mucoid Pseudomonas aeruginosa in patients with cystic fibrosis. Ann Pharmacother. 45(7-8):e38, 2011.

Varga K, Goldstein RF, Jurkuvenaite A, Chen L, Matalon S, Sorscher EJ, Bebok Z, Collawn JF. Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones. Biochemical Journal. 410 (3):555-64, 2008.

Virgin FW, Zhang S, Schuster D, Azbell C, Fortenberry J, Sorscher EJ, Woodworth BA.  The bioflavonoid compound, sinupret, stimulates transepithelial chloride transport in vitro and in vivo.  The Laryngoscope 120(5): 1051-1056, 2010.

Wang W, Okeyo GO, Tao B, Hong JS, Kirk KL. Thermally unstable gating of the most common cystic fibrosis mutant channel ({Delta}F508): 'Rescue' by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops. Journal of Biological Chemistry. Sep. 30, 2011. [Epub ahead of print]

Wang C, Protasevich I, Yang Z, Seehausen D, Skalak T, Zhao X, Atwell S, Spencer Emtage J, Wetmore DR, Brouillette CG, Hunt JF. Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis. Protein Science. 19(10):1932-47, 2010.

Wang W, Bernard K, Li G, Kirk KL. Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains. Journal of Biological Chemistry. 282(7): 4533-44, 2007.

Wang W, Wu J, Bernard K, Li G, Wang G, Bevensee MO, Kirk KL. ATP-independent CFTR channel gating and allosteric modulation by phosphorylation. Proceedings of the National Academy of USA. 23;107(8):3888-93, 2010.

Weathington NM, van Houwelingen AH, Noerager BD, Jackson PL, Kraneveld AD, Galin FS, Folkerts G, Nijkamp FP, Blalock JE. A novel peptide CXCR ligand derived from extracellular matrix degradation during airway inflammation. Nature Medicine. 12(3): 317-23, 2006.

White AF, Mazur M, Sorscher EJ, Zinn K, Ponnazhagan S           . Genetic modification of AAV2 capsid enhances gene transfer efficiency in polarized human airway epithelial cells. Human Gene Therapy. 19 (12):1407-14, 2008.

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Updated 4th Quarter, 2011