Search Results - "Wine, J"

Airway Gland Structure and Function by Widdicombe, Jonathan H, Wine, Jeffrey J

“…Submucosal glands contribute to airway surface liquid (ASL), a film that protects all airway surfaces. Glandular mucus comprises electrolytes, water, the…”
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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809 by Van Goor, Fredrick, Hadida, Sabine, Grootenhuis, Peter D. J, Burton, Bill, Stack, Jeffrey H, Straley, Kimberly S, Decker, Caroline J, Miller, Mark, McCartney, Jason, Olson, Eric R, Wine, Jeffrey J, Frizzell, Ray A, Ashlock, Melissa, Negulescu, Paul A

“…Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene that impair the function of CFTR, an epithelial chloride…”
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The normal trachea is cleaned by MUC5B mucin bundles from the submucosal glands coated with the MUC5AC mucin by Ermund, Anna, Meiss, Lauren N., Rodriguez-Pineiro, Ana M., Bähr, Andrea, Nilsson, Harriet E., Trillo-Muyo, Sergio, Ridley, Caroline, Thornton, David J., Wine, Jeffrey J., Hebert, Hans, Klymiuk, Nikolai, Hansson, Gunnar C.

“…To understand the mucociliary clearance system, mucins were visualized by light, confocal and electron microscopy, and mucus was stained by Alcian blue and…”
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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770 by Van Goor, Fredrick, Hadida, Sabine, Grootenhuis, Peter D.J, Burton, Bill, Cao, Dong, Neuberger, Tim, Turnbull, Amanda, Singh, Ashvani, Joubran, John, Hazlewood, Anna, Zhou, Jinglan, McCartney, Jason, Arumugam, Vijayalaksmi, Decker, Caroline, Yang, Jennifer, Young, Chris, Olson, Eric R, Wine, Jeffery J, Frizzell, Raymond A, Ashlock, Melissa, Negulescu, Paul

“…Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), a protein kinase A…”
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High-Efficiency, Selection-free Gene Repair in Airway Stem Cells from Cystic Fibrosis Patients Rescues CFTR Function in Differentiated Epithelia by Vaidyanathan, Sriram, Salahudeen, Ameen A., Sellers, Zachary M., Bravo, Dawn T., Choi, Shannon S., Batish, Arpit, Le, Wei, Baik, Ron, de la O, Sean, Kaushik, Milan P., Galper, Noah, Lee, Ciaran M., Teran, Christopher A., Yoo, Jessica H., Bao, Gang, Chang, Eugene H., Patel, Zara M., Hwang, Peter H., Wine, Jeffrey J., Milla, Carlos E., Desai, Tushar J., Nayak, Jayakar V., Kuo, Calvin J., Porteus, Matthew H.

“…Cystic fibrosis (CF) is a monogenic disorder caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Mortality in CF…”
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Granule-stored MUC5B mucins are packed by the non-covalent formation of N-terminal head-to-head tetramers by Trillo-Muyo, Sergio, Nilsson, Harriet E., Recktenwald, Christian V., Ermund, Anna, Ridley, Caroline, Meiss, Lauren N., Bähr, Andrea, Klymiuk, Nikolai, Wine, Jeffrey J., Koeck, Philip J.B., Thornton, David J., Hebert, Hans, Hansson, Gunnar C.

“…Most MUC5B mucin polymers in the upper airways of humans and pigs are produced by submucosal glands. MUC5B forms N-terminal covalent dimers that are further…”
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Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis by Sun, Xingshen, Sui, Hongshu, Fisher, John T, Yan, Ziying, Liu, Xiaoming, Cho, Hyung-Ju, Joo, Nam Soo, Zhang, Yulong, Zhou, Weihong, Yi, Yaling, Kinyon, Joann M, Lei-Butters, Diana C, Griffin, Michelle A, Naumann, Paul, Luo, Meihui, Ascher, Jill, Wang, Kai, Frana, Timothy, Wine, Jeffrey J, Meyerholz, David K, Engelhardt, John F

“…Cystic fibrosis (CF) is a recessive disease that affects multiple organs. It is caused by mutations in CFTR. Animal modeling of this disease has been…”
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Progress in understanding mucus abnormalities in cystic fibrosis airways by Wine, Jeffrey J., Hansson, Gunnar C., König, Peter, Joo, Nam Soo, Ermund, Anna, Pieper, Mario

“…Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via…”
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Variation in CFTR-dependent 'β-sweating' among healthy adults by DeRose, Lesje, Kim, Jeeyeon, Farahmand, Miesha, Shinbashi, Meagan Y, Joo, Nam Soo, Wine, Jeffrey J

“…The genetic disease cystic fibrosis (CF) results when mutations in the gene for the anion channel CFTR reduce CFTR's activity below a critical level. CFTR…”
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Parasympathetic control of airway submucosal glands: Central reflexes and the airway intrinsic nervous system by Wine, Jeffrey J

“…Abstract Airway submucosal glands produce the mucus that lines the upper airways to protect them against insults. This review summarizes evidence for two forms…”
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Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs by Joo, Nam Soo, Cho, Hyung-Ju, Khansaheb, Monal, Wine, Jeffrey J

“…Cystic fibrosis (CF) results from mutations that disrupt CF transmembrane conductance regulator (CFTR), an anion channel found mainly in apical membranes of…”
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Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model by Joo, Nam Soo, Cho, Hyung-Ju, Shinbashi, Meagan, Choi, Jae Young, Milla, Carlos E., Engelhardt, John F., Wine, Jeffrey J.

“…Mucus clearance, a primary innate defense mechanism of airways, is defective in patients with cystic fibrosis (CF) and CF animals. In previous work, the…”
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Evaporimeter and Bubble-Imaging Measures of Sweat Gland Secretion Rates by Kim, Jeeyeon, Farahmand, Miesha, Dunn, Colleen, Davies, Zoe, Frisbee, Eric, Milla, Carlos, Wine, Jeffrey J

“…Beta-adrenergically-stimulated sweat rates determined by evaporimetry or by sweat bubble imaging are useful for measuring CFTR function because they provide a…”
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Proteomic analysis of pure human airway gland mucus reveals a large component of protective proteins by Joo, Nam Soo, Evans, Idil Apak T, Cho, Hyung-Ju, Park, Il-Ho, Engelhardt, John F, Wine, Jeffrey J

“…Airway submucosal glands contribute to innate immunity and protect the lungs by secreting mucus, which is required for mucociliary clearance and which also…”
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The genesis of cystic fibrosis lung disease by Wine, J J

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In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands by Wine, Jeffrey J, Char, Jessica E, Chen, Jonathan, Cho, Hyung-Ju, Dunn, Colleen, Frisbee, Eric, Joo, Nam Soo, Milla, Carlos, Modlin, Sara E, Park, Il-Ho, Thomas, Ewart A C, Tran, Kim V, Verma, Rohan, Wolfe, Marlene H

“…To assess CFTR function in vivo, we developed a bioassay that monitors and compares CFTR-dependent and CFTR-independent sweat secretion in parallel for…”
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Lubiprostone stimulates secretion from tracheal submucosal glands of sheep, pigs, and humans by Joo, N S, Wine, J J, Cuthbert, A W

“…Lubiprostone, a putative ClC-2 chloride channel opener, has been investigated for its effects on airway epithelia (tracheas). Lubiprostone is shown to increase…”
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The development of lung disease in cystic fibrosis pigs by Wine, Jeffrey J

“…The leading cause of death in cystic fibrosis (CF) patients is lung disease; yet despite enormous research efforts, its pathogenesis is not well understood…”
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A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor by Char, Jessica E, Wolfe, Marlene H, Cho, Hyung-Ju, Park, Il-Ho, Jeong, Jin Hyeok, Frisbee, Eric, Dunn, Colleen, Davies, Zoe, Milla, Carlos, Moss, Richard B, Thomas, Ewart A C, Wine, Jeffrey J

“…To determine if oral dosing with the CFTR-potentiator ivacaftor (VX-770, Kalydeco) improves CFTR-dependent sweating in CF subjects carrying G551D or R117H-5T…”
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The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurements by Char, Jessica E, Dunn, Colleen, Davies, Zoe, Milla, Carlos, Moss, Richard B, Wine, Jeffrey J

“…We optically measured effects of orally available ivacaftor (Kalydeco®) on sweat rates of identified glands in 3 R117H subjects, each having a unique set of…”
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