CF Biomarker Consortium

Focus

The focus of the Cystic Fibrosis Biomarker Consortium is to identify biomarkers that:

• Track and predict disease progression (including early lung disease, lung function decline, and pulmonary exacerbations)
• Detect improved function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein for new candidate targets and pathways for CF
• Validate preclinical animal model systems

Consortium Membership  

Suggested Reading

• Clancy JP, Joseloff E. Take it to the bank. J Cyst Fibros. 2017 Sep;16(5):532-533.
• Muhlebach MS, Clancy JP, Heltshe SL, et al. Biomarkers for cystic fibrosis drug development. J Cyst Fibros. 2016 Oct;15(6):714-723.
• Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, Joseloff E, Ramsey BW; GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med. 2014 Jul 15;190(2):175-84.
• Heltshe SL, Rowe SM, Mayer-Hamblett N. Evaluating the predictive ability of sweat chloride. J Cyst Fibros. 2014 Jan;13(1):118.
• Locke LW, Myerburg MM, Markovetz MR, Parker RS, Weber L, Czachowski MR, Harding TJ, Brown SL, Nero JA, Pilewski JM, Corcoran TE. Quantitative imaging of airway liquid absorption in cystic fibrosis. Eur Respir J. 2014 Sep;44(3):675-84. 
• Accurso FJ, Van Goor F, Zha J, Stone AJ, Dong Q, Ordonez CL, Rowe SM, Clancy JP, Konstan MW, Hoch HE, Heltshe SL, Ramsey BW, Campbell PW, Ashlock MA. Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.J Cyst Fibros. 2014 Mar;13(2):139-47.
• Joseloff E, Sha W, Bell SC, Wetmore DR, Lawton KA, Milburn MV, Ryals JA, Guo L, Muhlebach MS. Serum metabolomics indicate altered cellular energy metabolism in children with cystic fibrosis. Pediatr Pulmonol. 2014 May;49(5):463-72.
• Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong Q, Ordoez CL, Stone AJ, Olson ER, Clancy JP; VX06-770-101 Study Group. Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation. PLoS One. 2013 Jul 26;8(7):e66955.
• Clancy JP, Szczesniak RD, Ashlock MA, Ernst SE, Fan L, Hornick DB, Karp PH, Khan U, Lymp J, Ostmann AJ, Rezayat A, Starner TD, Sugandha SP, Sun H, Quinney N, Donaldson SH, Rowe SM, Gabriel SE. Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function. PLoS One. 2013 Sep 10;8(9):e73905.
• Heltshe SL, Mayer-Hamblett N, Rowe SM. Understanding the relationship between sweat chloride and lung function in cystic fibrosis. Chest. 2013 Oct;144(4):1418. 
• Corcoran TE, Thomas KM, Brown S, Myerburg MM, Locke LW, Pilewski JM. Liquid hyper-absorption as a cause of increased DTPA clearance in the cystic fibrosis airway. EJNMMI Res. 2013 Feb 27;3(1):14. 
• Moss RB, Mistry SJ, Konstan MW, Pilewski JM, Kerem E, Tal-Singer R, Lazaar AL; CF2110399 Investigators. Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis. J Cyst Fibros. 2013 May;12(3):241-8.
• De Boeck K, Kent L, Davies J, Derichs N, Amaral M, Rowe SM, Middleton P, de Jonge H, Bronsveld I, Wilschanski M, Melotti P, Danner-Boucher I, Boerner S, Fajac I, Southern K, de Nooijer RA, Bot A, de Rijke Y, de Wachter E, Leal T, Vermeulen F, Hug MJ, Rault G, Nguyen-Khoa T, Barreto C, Proesmans M, Sermet-Gaudelus I; European Cystic Fibrosis Society Clinical Trial Network Standardisation Committee. CFTR biomarkers: time for promotion to surrogate end-point. Eur Respir J. 2013 Jan;41(1):203-16
• Rowe SM, Borowitz DS, Burns JL, Clancy JP, Donaldson SH, Retsch-Bogart G, Sagel SD, Ramsey BW. Progress in cystic fibrosis and the CF Therapeutics Development Network. Thorax. 2012 Oct;67(10):882-90.
• Sagel SD, Wagner BD, Anthony MM, Emmett P, Zemanick ET. Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis. Am J Respir Crit Care Med. 2012 Nov 1;186(9):857-65.
• Ratjen F, Saiman L, Mayer-Hamblett N, Lands LC, Kloster M, Thompson V, Emmett P, Marshall B, Accurso F, Sagel S, Anstead M. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest. 2012 Nov;142(5):1259-66.
• Ashlock MA, Olson ER. Therapeutics development for cystic fibrosis: a successful model for a multisystem genetic disease. Annu Rev Med. 2011 Feb 18;62:107-25. 
• Hug MJ, Derichs N, Bronsveld I, Clancy JP. Measurement of ion transport function in rectal biopsies. Methods Mol Biol. 2011;741:87-107. 
• Rowe SM, Clancy JP, Wilschanski M. Nasal potential difference measurements to assess CFTR ion channel activity. Methods Mol Biol. 2011;741:69-86.
• Zemanick ET, Sagel SD, Harris JK. The airway microbiome in cystic fibrosis and implications for treatment. Curr Opin Pediatr. 2011 Jun;23(3):319-24. 
• Wetmore DR, Joseloff E, Pilewski J, Lee DP, Lawton KA, Mitchell MW, Milburn MV, Ryals JA, Guo L. Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells. J Biol Chem. 2010 Oct 1;285(40):30516-22.