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Mucociliary Clearance in Mice Measured by Tracking Trans-tracheal Fluorescence of Nasally Aerosolized Beads.

Rogers TD, Ostrowski LE, Livraghi-Butrico A, Button B, Grubb BR.

Sci Rep. 2018 Oct 3;8(1):14744. doi: 10.1038/s41598-018-33053-2.


An Improved Inhaled Mucolytic to Treat Airway Muco-Obstructive Diseases.

Ehre C, Rushton ZL, Wang B, Hothem LN, Morrison CB, Fontana NC, Markovetz MR, Delion MF, Kato T, Villalon D, Thelin WR, Esther CR Jr, Hill DB, Grubb BR, Livraghi-Butrico A, Donaldson SH, Boucher RC.

Am J Respir Crit Care Med. 2018 Sep 13. doi: 10.1164/rccm.201802-0245OC. [Epub ahead of print]


Role of Spdef in the Regulation of Muc5b Expression in the Airways of Naive and Mucoobstructed Mice.

Chen G, Volmer AS, Wilkinson KJ, Deng Y, Jones LC, Yu D, Bustamante-Marin XM, Burns KA, Grubb BR, O'Neal WK, Livraghi-Butrico A, Boucher RC.

Am J Respir Cell Mol Biol. 2018 Sep;59(3):383-396. doi: 10.1165/rcmb.2017-0127OC.


Lung disease phenotypes caused by overexpression of combinations of α-, β-, and γ-subunits of the epithelial sodium channel in mouse airways.

Livraghi-Butrico A, Wilkinson KJ, Volmer AS, Gilmore RC, Rogers TD, Caldwell RA, Burns KA, Esther CR Jr, Mall MA, Boucher RC, O'Neal WK, Grubb BR.

Am J Physiol Lung Cell Mol Physiol. 2018 Feb 1;314(2):L318-L331. doi: 10.1152/ajplung.00382.2017. Epub 2017 Oct 26.


Identification of trans Protein QTL for Secreted Airway Mucins in Mice and a Causal Role for Bpifb1.

Donoghue LJ, Livraghi-Butrico A, McFadden KM, Thomas JM, Chen G, Grubb BR, O'Neal WK, Boucher RC, Kelada SNP.

Genetics. 2017 Oct;207(2):801-812. doi: 10.1534/genetics.117.300211. Epub 2017 Aug 29.


Contribution of mucus concentration and secreted mucins Muc5ac and Muc5b to the pathogenesis of muco-obstructive lung disease.

Livraghi-Butrico A, Grubb BR, Wilkinson KJ, Volmer AS, Burns KA, Evans CM, O'Neal WK, Boucher RC.

Mucosal Immunol. 2017 May;10(3):829. doi: 10.1038/mi.2017.29. No abstract available.


Contribution of mucus concentration and secreted mucins Muc5ac and Muc5b to the pathogenesis of muco-obstructive lung disease.

Livraghi-Butrico A, Grubb BR, Wilkinson KJ, Volmer AS, Burns KA, Evans CM, O'Neal WK, Boucher RC.

Mucosal Immunol. 2017 Mar;10(2):395-407. doi: 10.1038/mi.2016.63. Epub 2016 Jul 20.


Reduced mucociliary clearance in old mice is associated with a decrease in Muc5b mucin.

Grubb BR, Livraghi-Butrico A, Rogers TD, Yin W, Button B, Ostrowski LE.

Am J Physiol Lung Cell Mol Physiol. 2016 May 1;310(9):L860-7. doi: 10.1152/ajplung.00015.2016. Epub 2016 Mar 11.


Restoring ciliary function to differentiated primary ciliary dyskinesia cells with a lentiviral vector.

Ostrowski LE, Yin W, Patel M, Sechelski J, Rogers T, Burns K, Grubb BR, Olsen JC.

Gene Ther. 2014 Mar;21(3):253-61. doi: 10.1038/gt.2013.79. Epub 2014 Jan 23.


Muc5b is required for airway defence.

Roy MG, Livraghi-Butrico A, Fletcher AA, McElwee MM, Evans SE, Boerner RM, Alexander SN, Bellinghausen LK, Song AS, Petrova YM, Tuvim MJ, Adachi R, Romo I, Bordt AS, Bowden MG, Sisson JH, Woodruff PG, Thornton DJ, Rousseau K, De la Garza MM, Moghaddam SJ, Karmouty-Quintana H, Blackburn MR, Drouin SM, Davis CW, Terrell KA, Grubb BR, O'Neal WK, Flores SC, Cota-Gomez A, Lozupone CA, Donnelly JM, Watson AM, Hennessy CE, Keith RC, Yang IV, Barthel L, Henson PM, Janssen WJ, Schwartz DA, Boucher RC, Dickey BF, Evans CM.

Nature. 2014 Jan 16;505(7483):412-6. doi: 10.1038/nature12807. Epub 2013 Dec 8.


Loss of Cftr function exacerbates the phenotype of Na(+) hyperabsorption in murine airways.

Livraghi-Butrico A, Kelly EJ, Wilkinson KJ, Rogers TD, Gilmore RC, Harkema JR, Randell SH, Boucher RC, O'Neal WK, Grubb BR.

Am J Physiol Lung Cell Mol Physiol. 2013 Apr 1;304(7):L469-80. doi: 10.1152/ajplung.00150.2012. Epub 2013 Feb 1.


Overexpressing mouse model demonstrates the protective role of Muc5ac in the lungs.

Ehre C, Worthington EN, Liesman RM, Grubb BR, Barbier D, O'Neal WK, Sallenave JM, Pickles RJ, Boucher RC.

Proc Natl Acad Sci U S A. 2012 Oct 9;109(41):16528-33. doi: 10.1073/pnas.1206552109. Epub 2012 Sep 24. Erratum in: Proc Natl Acad Sci U S A. 2014 Apr 15;111(15):5753.


Regional differences in rat conjunctival ion transport activities.

Yu D, Thelin WR, Rogers TD, Stutts MJ, Randell SH, Grubb BR, Boucher RC.

Am J Physiol Cell Physiol. 2012 Oct 1;303(7):C767-80. doi: 10.1152/ajpcell.00195.2012. Epub 2012 Jul 18.


Genetically determined heterogeneity of lung disease in a mouse model of airway mucus obstruction.

Livraghi-Butrico A, Grubb BR, Kelly EJ, Wilkinson KJ, Yang H, Geiser M, Randell SH, Boucher RC, O'Neal WK.

Physiol Genomics. 2012 Apr 15;44(8):470-84. doi: 10.1152/physiolgenomics.00185.2011. Epub 2012 Mar 6.


Transgenic hCFTR expression fails to correct β-ENaC mouse lung disease.

Grubb BR, O'Neal WK, Ostrowski LE, Kreda SM, Button B, Boucher RC.

Am J Physiol Lung Cell Mol Physiol. 2012 Jan 15;302(2):L238-47. doi: 10.1152/ajplung.00083.2011. Epub 2011 Oct 14.


Human alveolar type II cells secrete and absorb liquid in response to local nucleotide signaling.

Bove PF, Grubb BR, Okada SF, Ribeiro CM, Rogers TD, Randell SH, O'Neal WK, Boucher RC.

J Biol Chem. 2010 Nov 5;285(45):34939-49. doi: 10.1074/jbc.M110.162933. Epub 2010 Aug 27.


Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice.

Mall MA, Button B, Johannesson B, Zhou Z, Livraghi A, Caldwell RA, Schubert SC, Schultz C, O'Neal WK, Pradervand S, Hummler E, Rossier BC, Grubb BR, Boucher RC.

J Biol Chem. 2010 Aug 27;285(35):26945-55. doi: 10.1074/jbc.M110.151803. Epub 2010 Jun 21.


Conditional deletion of dnaic1 in a murine model of primary ciliary dyskinesia causes chronic rhinosinusitis.

Ostrowski LE, Yin W, Rogers TD, Busalacchi KB, Chua M, O'Neal WK, Grubb BR.

Am J Respir Cell Mol Biol. 2010 Jul;43(1):55-63. doi: 10.1165/rcmb.2009-0118OC. Epub 2009 Aug 12.


Inactivation of Chibby affects function of motile airway cilia.

Voronina VA, Takemaru K, Treuting P, Love D, Grubb BR, Hajjar AM, Adams A, Li FQ, Moon RT.

J Cell Biol. 2009 Apr 20;185(2):225-33. doi: 10.1083/jcb.200809144. Epub 2009 Apr 13.


Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl- secretory channel in mouse airways.

Rock JR, O'Neal WK, Gabriel SE, Randell SH, Harfe BD, Boucher RC, Grubb BR.

J Biol Chem. 2009 May 29;284(22):14875-80. doi: 10.1074/jbc.C109.000869. Epub 2009 Apr 10.


Ion transport across CF and normal murine olfactory and ciliated epithelium.

Grubb BR, Rogers TD, Boucher RC, Ostrowski LE.

Am J Physiol Cell Physiol. 2009 Jun;296(6):C1301-9. doi: 10.1152/ajpcell.00578.2008. Epub 2009 Mar 25.


Airway and lung pathology due to mucosal surface dehydration in {beta}-epithelial Na+ channel-overexpressing mice: role of TNF-{alpha} and IL-4R{alpha} signaling, influence of neonatal development, and limited efficacy of glucocorticoid treatment.

Livraghi A, Grubb BR, Hudson EJ, Wilkinson KJ, Sheehan JK, Mall MA, O'Neal WK, Boucher RC, Randell SH.

J Immunol. 2009 Apr 1;182(7):4357-67. doi: 10.4049/jimmunol.0802557.


Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice.

Ostrowski LE, Yin W, Diggs PS, Rogers TD, O'Neal WK, Grubb BR.

Gene Ther. 2007 Oct;14(20):1492-501. Epub 2007 Jul 19.


Extracellular nucleotides block bone mineralization in vitro: evidence for dual inhibitory mechanisms involving both P2Y2 receptors and pyrophosphate.

Orriss IR, Utting JC, Brandao-Burch A, Colston K, Grubb BR, Burnstock G, Arnett TR.

Endocrinology. 2007 Sep;148(9):4208-16. Epub 2007 Jun 14.


Olfactory epithelia exhibit progressive functional and morphological defects in CF mice.

Grubb BR, Rogers TD, Kulaga HM, Burns KA, Wonsetler RL, Reed RR, Ostrowski LE.

Am J Physiol Cell Physiol. 2007 Aug;293(2):C574-83. Epub 2007 Apr 11.


SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis.

Grubb BR, Gabriel SE, Mengos A, Gentzsch M, Randell SH, Van Heeckeren AM, Knowles MR, Drumm ML, Riordan JR, Boucher RC.

Am J Respir Cell Mol Biol. 2006 Mar;34(3):355-63. Epub 2005 Nov 11.


The cystic fibrosis transmembrane conductance regulator is regulated by a direct interaction with the protein phosphatase 2A.

Thelin WR, Kesimer M, Tarran R, Kreda SM, Grubb BR, Sheehan JK, Stutts MJ, Milgram SL.

J Biol Chem. 2005 Dec 16;280(50):41512-20. Epub 2005 Oct 20.


Culture of murine nasal epithelia: model for cystic fibrosis.

Grubb BR, Rogers TD, Diggs PC, Boucher RC, Ostrowski LE.

Am J Physiol Lung Cell Mol Physiol. 2006 Feb;290(2):L270-7. Epub 2005 Sep 9.


Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces.

Matsui H, Verghese MW, Kesimer M, Schwab UE, Randell SH, Sheehan JK, Grubb BR, Boucher RC.

J Immunol. 2005 Jul 15;175(2):1090-9.


Nucleotide release provides a mechanism for airway surface liquid homeostasis.

Lazarowski ER, Tarran R, Grubb BR, van Heusden CA, Okada S, Boucher RC.

J Biol Chem. 2004 Aug 27;279(35):36855-64. Epub 2004 Jun 21.


Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice.

Mall M, Grubb BR, Harkema JR, O'Neal WK, Boucher RC.

Nat Med. 2004 May;10(5):487-93. Epub 2004 Apr 11.


Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium.

Coakley RD, Grubb BR, Paradiso AM, Gatzy JT, Johnson LG, Kreda SM, O'Neal WK, Boucher RC.

Proc Natl Acad Sci U S A. 2003 Dec 23;100(26):16083-8. Epub 2003 Dec 10.


Mucociliary transport determined by in vivo microdialysis in the airways of normal and CF mice.

Grubb BR, Jones JH, Boucher RC.

Am J Physiol Lung Cell Mol Physiol. 2004 Mar;286(3):L588-95. Epub 2003 Nov 21.


Expression of the amino acid transporter ATB 0+ in lung: possible role in luminal protein removal.

Sloan JL, Grubb BR, Mager S.

Am J Physiol Lung Cell Mol Physiol. 2003 Jan;284(1):L39-49. Epub 2002 Aug 23.


In vivo microdialysis for determination of nasal liquid ion composition.

Grubb BR, Chadburn JL, Boucher RC.

Am J Physiol Cell Physiol. 2002 Jun;282(6):C1423-31.


Bioelectric measurement of CFTR function in mice.

Grubb BR.

Methods Mol Med. 2002;70:525-35. No abstract available.


In vivo airway surface liquid Cl- analysis with solid-state electrodes.

Caldwell RA, Grubb BR, Tarran R, Boucher RC, Knowles MR, Barker PM.

J Gen Physiol. 2002 Jan;119(1):3-14.


Isolation and culture of airway epithelial cells from chronically infected human lungs.

Randell SH, Walstad L, Schwab UE, Grubb BR, Yankaskas JR.

In Vitro Cell Dev Biol Anim. 2001 Sep;37(8):480-9.


The CF salt controversy: in vivo observations and therapeutic approaches.

Tarran R, Grubb BR, Parsons D, Picher M, Hirsh AJ, Davis CW, Boucher RC.

Mol Cell. 2001 Jul;8(1):149-58.


Alterations in airway ion transport in NKCC1-deficient mice.

Grubb BR, Pace AJ, Lee E, Koller BH, Boucher RC.

Am J Physiol Cell Physiol. 2001 Aug;281(2):C615-23.


Cloning and functional characterization of two murine uridine nucleotide receptors reveal a potential target for correcting ion transport deficiency in cystic fibrosis gallbladder.

Lazarowski ER, Rochelle LG, O'Neal WK, Ribeiro CM, Grubb BR, Zhang V, Harden TK, Boucher RC.

J Pharmacol Exp Ther. 2001 Apr;297(1):43-9.


Intestinal ion transport in NKCC1-deficient mice.

Grubb BR, Lee E, Pace AJ, Koller BH, Boucher RC.

Am J Physiol Gastrointest Liver Physiol. 2000 Oct;279(4):G707-18.


Effect of loss of P2Y(2) receptor gene expression on nucleotide regulation of murine epithelial Cl(-) transport.

Cressman VL, Lazarowski E, Homolya L, Boucher RC, Koller BH, Grubb BR.

J Biol Chem. 1999 Sep 10;274(37):26461-8.


Ion transport across the normal and CF neonatal murine intestine.

Grubb BR.

Am J Physiol. 1999 Jul;277(1 Pt 1):G167-74.


Salt restriction induces pseudohypoaldosteronism type 1 in mice expressing low levels of the beta-subunit of the amiloride-sensitive epithelial sodium channel.

Pradervand S, Barker PM, Wang Q, Ernst SA, Beermann F, Grubb BR, Burnier M, Schmidt A, Bindels RJ, Gatzy JT, Rossier BC, Hummler E.

Proc Natl Acad Sci U S A. 1999 Feb 16;96(4):1732-7.


Pathophysiology of gene-targeted mouse models for cystic fibrosis.

Grubb BR, Boucher RC.

Physiol Rev. 1999 Jan;79(1 Suppl):S193-214. Review.


Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease.

Matsui H, Grubb BR, Tarran R, Randell SH, Gatzy JT, Davis CW, Boucher RC.

Cell. 1998 Dec 23;95(7):1005-15.


Enhanced in vivo airway gene transfer via transient modification of host barrier properties with a surface-active agent.

Parsons DW, Grubb BR, Johnson LG, Boucher RC.

Hum Gene Ther. 1998 Dec 10;9(18):2661-72.


Effect of in vivo corticosteroids on Na+ transport across airway epithelia.

Grubb BR, Boucher RC.

Am J Physiol. 1998 Jul;275(1 Pt 1):C303-8.


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