Search Results - "Rowe, Steven M."

Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist by Nichols, David P, Morgan, Sarah J, Skalland, Michelle, Vo, Anh T, Van Dalfsen, Jill M, Singh, Sachinkumar Bp, Ni, Wendy, Hoffman, Lucas R, McGeer, Kailee, Heltshe, Sonya L, Clancy, John P, Rowe, Steven M, Jorth, Peter, Singh, Pradeep K

“…BackgroundLung infections are among the most consequential manifestations of cystic fibrosis (CF) and are associated with reduced lung function and shortened…”
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Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice by Hancock, Laura A., Hennessy, Corinne E., Solomon, George M., Dobrinskikh, Evgenia, Estrella, Alani, Hara, Naoko, Hill, David B., Kissner, William J., Markovetz, Matthew R., Grove Villalon, Diane E., Voss, Matthew E., Tearney, Guillermo J., Carroll, Kate S., Shi, Yunlong, Schwarz, Marvin I., Thelin, William R., Rowe, Steven M., Yang, Ivana V., Evans, Christopher M., Schwartz, David A.

“…The gain-of-function MUC5B promoter variant rs35705950 is the dominant risk factor for developing idiopathic pulmonary fibrosis (IPF). Here we show in humans…”
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The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis by Shei, Ren-Jay, Peabody, Jacelyn E, Kaza, Niroop, Rowe, Steven M

“…•ENaC contributes to airway surface liquid homeostasis in CF, though whether ENaC activity is abnormal in CF is unresolved.•ENaC inhibitors are in development…”
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Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression by Roy, Bijoyita, Friesen, Westley J., Tomizawa, Yuki, Leszyk, John D., Zhuo, Jin, Johnson, Briana, Dakka, Jumana, Trotta, Christopher R., Xue, Xiaojiao, Mutyam, Venkateshwar, Keeling, Kim M., Mobley, James A., Rowe, Steven M., Bedwell, David M., Welch, Ellen M., Jacobson, Allan

“…A premature termination codon (PTC) in the ORF of an mRNA generally leads to production of a truncated polypeptide, accelerated degradation of the mRNA, and…”
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Cigarette smoke and CFTR: implications in the pathogenesis of COPD by Rab, Andras, Rowe, Steven M, Raju, S Vamsee, Bebok, Zsuzsa, Matalon, Sadis, Collawn, James F

“…Chronic obstructive pulmonary disease (COPD) is a progressive respiratory disorder consisting of chronic bronchitis and/or emphysema. COPD patients suffer from…”
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Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in the Lower Airways in COPD by Dransfield, Mark T., MD, Wilhelm, Andrew M., MD, Flanagan, Brian, MD, Courville, Clifford, MD, Tidwell, Sherry L., RRT, Raju, S. Vamsee, PhD, Gaggar, Amit, MD, PhD, Steele, Chad, PhD, Tang, Li Ping, DVM, Liu, Bo, MD, Rowe, Steven M., MD, MSPH

“…Background Cigarette smoke and smoking-induced inflammation decrease cystic fibrosis transmembrane conductance regulator (CFTR) activity and mucociliary…”
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An ex vivo rat trachea model reveals abnormal airway physiology and a gland secretion defect in cystic fibrosis by Harris, Elex, Easter, Molly, Ren, Janna, Krick, Stefanie, Barnes, Jarrod, Rowe, Steven M

“…Cystic fibrosis (CF) is a genetic disease hallmarked by aberrant ion transport that results in delayed mucus clearance, chronic infection, and progressive lung…”
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A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease by Sloane, Peter A, Shastry, Suresh, Wilhelm, Andrew, Courville, Clifford, Tang, Li Ping, Backer, Kyle, Levin, Elina, Raju, S Vamsee, Li, Yao, Mazur, Marina, Byan-Parker, Suzanne, Grizzle, William, Sorscher, Eric J, Dransfield, Mark T, Rowe, Steven M

“…Mucus stasis in chronic obstructive pulmonary disease (COPD) is a significant contributor to morbidity and mortality. Potentiators of cystic fibrosis…”
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A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial by Boyle, Michael P, Dr, Bell, Scott C, Prof, Konstan, Michael W, Prof, McColley, Susanna A, Prof, Rowe, Steven M, MD, Rietschel, Ernst, Prof, Huang, Xiaohong, PhD, Waltz, David, MD, Patel, Naimish R, MD, Rodman, David, MD

“…Summary Background The phe508del CFTR mutation causes cystic fibrosis by limiting the amount of CFTR protein that reaches the epithelial cell surface. We…”
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Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography by Liu, Linbo, Chu, Kengyeh K, Houser, Grace H, Diephuis, Bradford J, Li, Yao, Wilsterman, Eric J, Shastry, Suresh, Dierksen, Gregory, Birket, Susan E, Mazur, Marina, Byan-Parker, Suzanne, Grizzle, William E, Sorscher, Eric J, Rowe, Steven M, Tearney, Guillermo J

“…We demonstrate the use of a high resolution form of optical coherence tomography, termed micro-OCT (μOCT), for investigating the functional microanatomy of…”
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Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial by Moss, Richard B, Dr Prof, Flume, Patrick A, Prof, Elborn, J Stuart, Prof, Cooke, Jon, MSc, Rowe, Steven M, MD, McColley, Susanna A, MD, Rubenstein, Ronald C, MD, Higgins, Mark, MD

“…Summary Background Ivacaftor has been previously assessed in patients with cystic fibrosis with Gly551Asp-CFTR or other gating mutations. We assessed ivacaftor…”
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The therapeutic potential of CFTR modulators for COPD and other airway diseases by Solomon, George M, Fu, Lianwu, Rowe, Steven M, Collawn, James F

“…•Common airway diseases including COPD, asthma and non-CF bronchiectasis (NCFBE) share the common pathophysiologic feature of mucus obstruction.•Acquired CFTR…”
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The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report by Johns, J Dixon, Rowe, Steven M

“…Cystic fibrosis (CF) is a genetic disorder of the epithelial CFTR apical chloride channel resulting in multi-organ manifestations, including pancreatic…”
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Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats by Tuggle, Katherine L, Birket, Susan E, Cui, Xiaoxia, Hong, Jeong, Warren, Joe, Reid, Lara, Chambers, Andre, Ji, Diana, Gamber, Kevin, Chu, Kengyeh K, Tearney, Guillermo, Tang, Li Ping, Fortenberry, James A, Du, Ming, Cadillac, Joan M, Bedwell, David M, Rowe, Steven M, Sorscher, Eric J, Fanucchi, Michelle V

“…Animal models for cystic fibrosis (CF) have contributed significantly to our understanding of disease pathogenesis. Here we describe development and…”
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Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR by Birket, Susan E, Davis, Joy M, Fernandez-Petty, Courtney M, Henderson, Alexander G, Oden, Ashley M, Tang, LiPing, Wen, Hui, Hong, Jeong, Fu, Lianwu, Chambers, Andre, Fields, Alvin, Zhao, Gojun, Tearney, Guillermo J, Sorscher, Eric J, Rowe, Steven M

“…Animal models have been highly informative for understanding the characteristics, onset, and progression of cystic fibrosis (CF) lung disease. In particular,…”
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Prime editing-mediated correction of the CFTR W1282X mutation in iPSCs and derived airway epithelial cells by Li, Chao, Liu, Zhong, Anderson, Justin, Liu, Zhongyu, Tang, Liping, Li, Yao, Peng, Ning, Chen, Jianguo, Liu, Xueming, Fu, Lianwu, Townes, Tim M, Rowe, Steven M, Bedwell, David M, Guimbellot, Jennifer, Zhao, Rui

“…A major unmet need in the cystic fibrosis (CF) therapeutic landscape is the lack of effective treatments for nonsense CFTR mutations, which affect…”
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A Critical Role for LTA₄H in Limiting Chronic Pulmonary Neutrophilic Inflammation by Snelgrove, Robert J., Jackson, Patricia L., Hardison, Matthew T., Noerager, Brett D., Kinloch, Andrew, Gaggar, Amit, Shastry, Suresh, Rowe, Steven M., Shim, Yun M., Hussell, Tracy, Blalock, J. Edwin

“…Leukotriene A₄ hydrolase (LTA₄H) is a proinflammatory enzyme that generates the inflammatory mediator leukotriene B₄ (LTB₄). LTA₄H also possesses…”
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Reduced sialylation of airway mucin impairs mucus transport by altering the biophysical properties of mucin by Harris, Elex S., McIntire, Hannah J., Mazur, Marina, Schulz-Hildebrandt, Hinnerk, Leung, Hui Min, Tearney, Guillermo J., Krick, Stefanie, Rowe, Steven M., Barnes, Jarrod W.

“…Mucus stasis is a pathologic hallmark of muco-obstructive diseases, including cystic fibrosis (CF). Mucins, the principal component of mucus, are extensively…”
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Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor by Xue, Xiaojiao, Mutyam, Venkateshwar, Tang, Liping, Biswas, Silpak, Du, Ming, Jackson, Laura A, Dai, Yanying, Belakhov, Valery, Shalev, Moran, Chen, Fuquan, Schacht, Jochen, J Bridges, Robert, Baasov, Timor, Hong, Jeong, Bedwell, David M, Rowe, Steven M

“…New drugs are needed to enhance premature termination codon (PTC) suppression to treat the underlying cause of cystic fibrosis (CF) and other diseases caused…”
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Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study by Zouk, Aline N, Gulati, Swati, Xing, Dongqi, Wille, Keith M, Rowe, Steven M, Wells, J Michael

“…Pulmonary artery (PA) enlargement, defined as pulmonary artery to ascending aorta diameter ratio (PA:A)>1 on computed tomography (CT), is a marker of pulmonary…”
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