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Items: 1 to 50 of 488

1.

Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.

Alaiwa MHA, Launspach JL, Grogan B, Carter S, Zabner J, Stoltz DA, Singh PK, McKone EF, Welsh MJ.

JCI Insight. 2018 Aug 9;3(15). pii: 121468. doi: 10.1172/jci.insight.121468. [Epub ahead of print]

2.

The vagal ganglia transcriptome identifies candidate therapeutics for airway hyperreactivity.

Reznikov LR, Meyerholz DK, Abou Alaiwa M, Kuan SP, Liao YJ, Bormann NL, Bair TB, Price M, Stoltz DA, Welsh MJ.

Am J Physiol Lung Cell Mol Physiol. 2018 Aug 1;315(2):L133-L148. doi: 10.1152/ajplung.00557.2017. Epub 2018 Apr 5.

3.

Small heat shock protein speciation: novel non-canonical 44 kDa HspB5-related protein species in rat and human tissues.

Benndorf R, Gilmont RR, Hirano S, Ransom RF, Jungblut PR, Bommer M, Goldman JE, Welsh MJ.

Cell Stress Chaperones. 2018 Mar 14. doi: 10.1007/s12192-018-0890-5. [Epub ahead of print]

PMID:
29542021
4.

Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.

Meyerholz DK, Stoltz DA, Gansemer ND, Ernst SE, Cook DP, Strub MD, LeClair EN, Barker CK, Adam RJ, Leidinger MR, Gibson-Corley KN, Karp PH, Welsh MJ, McCray PB Jr.

Lab Invest. 2018 Jun;98(6):825-838. doi: 10.1038/s41374-018-0026-7. Epub 2018 Feb 21.

PMID:
29467455
5.

Nominal carbonic anhydrase activity minimizes airway-surface liquid pH changes during breathing.

Thornell IM, Li X, Tang XX, Brommel CM, Karp PH, Welsh MJ, Zabner J.

Physiol Rep. 2018 Jan;6(2). doi: 10.14814/phy2.13569.

6.

Motile cilia of human airway epithelia contain hedgehog signaling components that mediate noncanonical hedgehog signaling.

Mao S, Shah AS, Moninger TO, Ostedgaard LS, Lu L, Tang XX, Thornell IM, Reznikov LR, Ernst SE, Karp PH, Tan P, Keshavjee S, Abou Alaiwa MH, Welsh MJ.

Proc Natl Acad Sci U S A. 2018 Feb 6;115(6):1370-1375. doi: 10.1073/pnas.1719177115. Epub 2018 Jan 22.

7.

Transient acidosis while retrieving a fear-related memory enhances its lability.

Du J, Price MP, Taugher RJ, Grigsby D, Ash JJ, Stark AC, Hossain Saad MZ, Singh K, Mandal J, Wemmie JA, Welsh MJ.

Elife. 2017 Jun 26;6. pii: e22564. doi: 10.7554/eLife.22564.

8.

Postnatal airway growth in cystic fibrosis piglets.

Adam RJ, Abou Alaiwa MH, Bouzek DC, Cook DP, Gansemer ND, Taft PJ, Powers LS, Stroik MR, Hoegger MJ, McMenimen JD, Hoffman EA, Zabner J, Welsh MJ, Meyerholz DK, Stoltz DA.

J Appl Physiol (1985). 2017 Sep 1;123(3):526-533. doi: 10.1152/japplphysiol.00263.2017. Epub 2017 Jun 15.

PMID:
28620056
9.

Gel-forming mucins form distinct morphologic structures in airways.

Ostedgaard LS, Moninger TO, McMenimen JD, Sawin NM, Parker CP, Thornell IM, Powers LS, Gansemer ND, Bouzek DC, Cook DP, Meyerholz DK, Abou Alaiwa MH, Stoltz DA, Welsh MJ.

Proc Natl Acad Sci U S A. 2017 Jun 27;114(26):6842-6847. doi: 10.1073/pnas.1703228114. Epub 2017 Jun 12.

10.

AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.

Ramsey BW, Welsh MJ.

Am J Respir Crit Care Med. 2017 May 1;195(9):1092-1099. doi: 10.1164/rccm.201702-0266ED. No abstract available.

11.

Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.

Hisert KB, Heltshe SL, Pope C, Jorth P, Wu X, Edwards RM, Radey M, Accurso FJ, Wolter DJ, Cooke G, Adam RJ, Carter S, Grogan B, Launspach JL, Donnelly SC, Gallagher CG, Bruce JE, Stoltz DA, Welsh MJ, Hoffman LR, McKone EF, Singh PK.

Am J Respir Crit Care Med. 2017 Jun 15;195(12):1617-1628. doi: 10.1164/rccm.201609-1954OC.

12.

Acid-Sensing Ion Channel 1a Contributes to Airway Hyperreactivity in Mice.

Reznikov LR, Meyerholz DK, Adam RJ, Abou Alaiwa M, Jaffer O, Michalski AS, Powers LS, Price MP, Stoltz DA, Welsh MJ.

PLoS One. 2016 Nov 7;11(11):e0166089. doi: 10.1371/journal.pone.0166089. eCollection 2016.

13.

CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes.

Steines B, Dickey DD, Bergen J, Excoffon KJ, Weinstein JR, Li X, Yan Z, Abou Alaiwa MH, Shah VS, Bouzek DC, Powers LS, Gansemer ND, Ostedgaard LS, Engelhardt JF, Stoltz DA, Welsh MJ, Sinn PL, Schaffer DV, Zabner J.

JCI Insight. 2016 Sep 8;1(14):e88728.

14.

Lentiviral-mediated phenotypic correction of cystic fibrosis pigs.

Cooney AL, Abou Alaiwa MH, Shah VS, Bouzek DC, Stroik MR, Powers LS, Gansemer ND, Meyerholz DK, Welsh MJ, Stoltz DA, Sinn PL, McCray PB Jr.

JCI Insight. 2016 Sep 8;1(14). pii: e88730.

15.

Repurposing tromethamine as inhaled therapy to treat CF airway disease.

Abou Alaiwa MH, Launspach JL, Sheets KA, Rivera JA, Gansemer ND, Taft PJ, Thorne PS, Welsh MJ, Stoltz DA, Zabner J.

JCI Insight. 2016 Jun 2;1(8). pii: e87535.

16.

Acute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalities.

Adam RJ, Hisert KB, Dodd JD, Grogan B, Launspach JL, Barnes JK, Gallagher CG, Sieren JP, Gross TJ, Fischer AJ, Cavanaugh JE, Hoffman EA, Singh PK, Welsh MJ, McKone EF, Stoltz DA.

JCI Insight. 2016 Apr 7;1(4):e86183.

17.

Relationships among CFTR expression, HCO3- secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies.

Shah VS, Ernst S, Tang XX, Karp PH, Parker CP, Ostedgaard LS, Welsh MJ.

Proc Natl Acad Sci U S A. 2016 May 10;113(19):5382-7. doi: 10.1073/pnas.1604905113. Epub 2016 Apr 25.

18.

Ivacaftor-Induced Proteomic Changes Suggest Monocyte Defects May Contribute to the Pathogenesis of Cystic Fibrosis.

Hisert KB, Schoenfelt KQ, Cooke G, Grogan B, Launspach JL, Gallagher CG, Donnelly SC, Welsh MJ, Singh PK, McKone EF, Becker L.

Am J Respir Cell Mol Biol. 2016 Apr;54(4):594-7. doi: 10.1165/rcmb.2015-0322LE. No abstract available.

19.

Newborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus.

Bartlett JA, Ramachandran S, Wohlford-Lenane CL, Barker CK, Pezzulo AA, Zabner J, Welsh MJ, Meyerholz DK, Stoltz DA, McCray PB Jr.

Am J Respir Crit Care Med. 2016 Oct 1;194(7):845-854.

20.

Airway acidification initiates host defense abnormalities in cystic fibrosis mice.

Shah VS, Meyerholz DK, Tang XX, Reznikov L, Abou Alaiwa M, Ernst SE, Karp PH, Wohlford-Lenane CL, Heilmann KP, Leidinger MR, Allen PD, Zabner J, McCray PB Jr, Ostedgaard LS, Stoltz DA, Randak CO, Welsh MJ.

Science. 2016 Jan 29;351(6272):503-7. doi: 10.1126/science.aad5589.

21.

Acidic pH increases airway surface liquid viscosity in cystic fibrosis.

Tang XX, Ostedgaard LS, Hoegger MJ, Moninger TO, Karp PH, McMenimen JD, Choudhury B, Varki A, Stoltz DA, Welsh MJ.

J Clin Invest. 2016 Mar 1;126(3):879-91. doi: 10.1172/JCI83922. Epub 2016 Jan 25.

22.

Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.

Li X, Tang XX, Vargas Buonfiglio LG, Comellas AP, Thornell IM, Ramachandran S, Karp PH, Taft PJ, Sheets K, Abou Alaiwa MH, Welsh MJ, Meyerholz DK, Stoltz DA, Zabner J.

Am J Physiol Lung Cell Mol Physiol. 2016 Apr 1;310(7):L670-9. doi: 10.1152/ajplung.00422.2015. Epub 2016 Jan 22.

23.

Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans.

Meyerholz DK, Lambertz AM, Reznikov LR, Ofori-Amanfo GK, Karp PH, McCray PB Jr, Welsh MJ, Stoltz DA.

Toxicol Pathol. 2016 Apr;44(3):434-41. doi: 10.1177/0192623315609691. Epub 2015 Oct 27.

24.

Breathing Inhibited When Seizures Spread to the Amygdala and upon Amygdala Stimulation.

Dlouhy BJ, Gehlbach BK, Kreple CJ, Kawasaki H, Oya H, Buzza C, Granner MA, Welsh MJ, Howard MA, Wemmie JA, Richerson GB.

J Neurosci. 2015 Jul 15;35(28):10281-9. doi: 10.1523/JNEUROSCI.0888-15.2015.

25.

New blood: Creative funding of disease-specific research.

Seaman WE, Locksley RM, Welsh MJ.

Sci Transl Med. 2015 May 20;7(288):288ed5. doi: 10.1126/scitranslmed.aaa9513. No abstract available.

PMID:
25995219
27.

Origins of cystic fibrosis lung disease.

Stoltz DA, Meyerholz DK, Welsh MJ.

N Engl J Med. 2015 Apr 16;372(16):1574-5. doi: 10.1056/NEJMc1502191. No abstract available.

28.

Origins of cystic fibrosis lung disease.

Stoltz DA, Meyerholz DK, Welsh MJ.

N Engl J Med. 2015 Jan 22;372(4):351-62. doi: 10.1056/NEJMra1300109. Review. No abstract available.

29.

pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37.

Abou Alaiwa MH, Reznikov LR, Gansemer ND, Sheets KA, Horswill AR, Stoltz DA, Zabner J, Welsh MJ.

Proc Natl Acad Sci U S A. 2014 Dec 30;111(52):18703-8. doi: 10.1073/pnas.1422091112. Epub 2014 Dec 15.

30.

Expression and activity of acid-sensing ion channels in the mouse anterior pituitary.

Du J, Reznikov LR, Welsh MJ.

PLoS One. 2014 Dec 15;9(12):e115310. doi: 10.1371/journal.pone.0115310. eCollection 2014.

31.

Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor.

Chang EH, Tang XX, Shah VS, Launspach JL, Ernst SE, Hilkin B, Karp PH, Abou Alaiwa MH, Graham SM, Hornick DB, Welsh MJ, Stoltz DA, Zabner J.

Int Forum Allergy Rhinol. 2015 Feb;5(2):178-81. doi: 10.1002/alr.21440. Epub 2014 Oct 31.

32.

Neuronal necrosis is regulated by a conserved chromatin-modifying cascade.

Liu K, Ding L, Li Y, Yang H, Zhao C, Lei Y, Han S, Tao W, Miao D, Steller H, Welsh MJ, Liu L.

Proc Natl Acad Sci U S A. 2014 Sep 23;111(38):13960-5. doi: 10.1073/pnas.1413644111. Epub 2014 Sep 8.

33.

Renal denervation using focused infrared fiber lasers: a potential treatment for hypertension.

Alexander VV, Shi Z, Iftekher F, Welsh MJ, Gurm HS, Rising G, Yanovich A, Walacavage K, Islam MN.

Lasers Surg Med. 2014 Nov;46(9):689-702. doi: 10.1002/lsm.22290. Epub 2014 Aug 29.

34.

Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass.

Uc A, Olivier AK, Griffin MA, Meyerholz DK, Yao J, Abu-El-Haija M, Buchanan KM, Vanegas Calderón OG, Abu-El-Haija M, Pezzulo AA, Reznikov LR, Hoegger MJ, Rector MV, Ostedgaard LS, Taft PJ, Gansemer ND, Ludwig PS, Hornick EE, Stoltz DA, Ode KL, Welsh MJ, Engelhardt JF, Norris AW.

Clin Sci (Lond). 2015 Jan;128(2):131-42. doi: 10.1042/CS20140059.

35.

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis.

Hoegger MJ, Fischer AJ, McMenimen JD, Ostedgaard LS, Tucker AJ, Awadalla MA, Moninger TO, Michalski AS, Hoffman EA, Zabner J, Stoltz DA, Welsh MJ.

Science. 2014 Aug 15;345(6198):818-22. doi: 10.1126/science.1255825.

36.

Acid-sensing ion channels contribute to synaptic transmission and inhibit cocaine-evoked plasticity.

Kreple CJ, Lu Y, Taugher RJ, Schwager-Gutman AL, Du J, Stump M, Wang Y, Ghobbeh A, Fan R, Cosme CV, Sowers LP, Welsh MJ, Radley JJ, LaLumiere RT, Wemmie JA.

Nat Neurosci. 2014 Aug;17(8):1083-91. doi: 10.1038/nn.3750. Epub 2014 Jun 22.

37.

Protons are a neurotransmitter that regulates synaptic plasticity in the lateral amygdala.

Du J, Reznikov LR, Price MP, Zha XM, Lu Y, Moninger TO, Wemmie JA, Welsh MJ.

Proc Natl Acad Sci U S A. 2014 Jun 17;111(24):8961-6. doi: 10.1073/pnas.1407018111. Epub 2014 Jun 2.

38.

A genomic signature approach to rescue ΔF508-cystic fibrosis transmembrane conductance regulator biosynthesis and function.

Ramachandran S, Osterhaus SR, Karp PH, Welsh MJ, McCray PB Jr.

Am J Respir Cell Mol Biol. 2014 Sep;51(3):354-62. doi: 10.1165/rcmb.2014-0007OC.

39.

Antibacterial properties of the CFTR potentiator ivacaftor.

Reznikov LR, Abou Alaiwa MH, Dohrn CL, Gansemer ND, Diekema DJ, Stoltz DA, Welsh MJ.

J Cyst Fibros. 2014 Sep;13(5):515-9. doi: 10.1016/j.jcf.2014.02.004. Epub 2014 Mar 5.

40.

Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs.

Hoegger MJ, Awadalla M, Namati E, Itani OA, Fischer AJ, Tucker AJ, Adam RJ, McLennan G, Hoffman EA, Stoltz DA, Welsh MJ.

Proc Natl Acad Sci U S A. 2014 Feb 11;111(6):2355-60. doi: 10.1073/pnas.1323633111. Epub 2014 Jan 28.

41.

Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study.

Abou Alaiwa MH, Beer AM, Pezzulo AA, Launspach JL, Horan RA, Stoltz DA, Starner TD, Welsh MJ, Zabner J.

J Cyst Fibros. 2014 Jul;13(4):373-7. doi: 10.1016/j.jcf.2013.12.006. Epub 2014 Jan 11.

42.

Localization and behaviors in null mice suggest that ASIC1 and ASIC2 modulate responses to aversive stimuli.

Price MP, Gong H, Parsons MG, Kundert JR, Reznikov LR, Bernardinelli L, Chaloner K, Buchanan GF, Wemmie JA, Richerson GB, Cassell MD, Welsh MJ.

Genes Brain Behav. 2014 Feb;13(2):179-94. doi: 10.1111/gbb.12108. Epub 2013 Dec 30.

43.

Chemosensory functions for pulmonary neuroendocrine cells.

Gu X, Karp PH, Brody SL, Pierce RA, Welsh MJ, Holtzman MJ, Ben-Shahar Y.

Am J Respir Cell Mol Biol. 2014 Mar;50(3):637-46. doi: 10.1165/rcmb.2013-0199OC.

44.

Loss of Acid sensing ion channel-1a and bicarbonate administration attenuate the severity of traumatic brain injury.

Yin T, Lindley TE, Albert GW, Ahmed R, Schmeiser PB, Grady MS, Howard MA, Welsh MJ.

PLoS One. 2013 Aug 26;8(8):e72379. doi: 10.1371/journal.pone.0072379. eCollection 2013.

45.

ATP and AMP mutually influence their interaction with the ATP-binding cassette (ABC) adenylate kinase cystic fibrosis transmembrane conductance regulator (CFTR) at separate binding sites.

Randak CO, Dong Q, Ver Heul AR, Elcock AH, Welsh MJ.

J Biol Chem. 2013 Sep 20;288(38):27692-701. doi: 10.1074/jbc.M113.479675. Epub 2013 Aug 6.

46.

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs.

Stoltz DA, Rokhlina T, Ernst SE, Pezzulo AA, Ostedgaard LS, Karp PH, Samuel MS, Reznikov LR, Rector MV, Gansemer ND, Bouzek DC, Abou Alaiwa MH, Hoegger MJ, Ludwig PS, Taft PJ, Wallen TJ, Wohlford-Lenane C, McMenimen JD, Chen JH, Bogan KL, Adam RJ, Hornick EE, Nelson GA 4th, Hoffman EA, Chang EH, Zabner J, McCray PB Jr, Prather RS, Meyerholz DK, Welsh MJ.

J Clin Invest. 2013 Jun;123(6):2685-93. doi: 10.1172/JCI68867. Epub 2013 May 8.

47.

Computational identification of operon-like transcriptional loci in eukaryotes.

Nannapaneni K, Ben-Shahar Y, Keen HL, Welsh MJ, Casavant TL, Scheetz TE.

Comput Biol Med. 2013 Jul;43(6):738-43. doi: 10.1016/j.compbiomed.2013.03.004. Epub 2013 Mar 28.

PMID:
23668349
48.

Post-transcriptional regulation of cystic fibrosis transmembrane conductance regulator expression and function by microRNAs.

Ramachandran S, Karp PH, Osterhaus SR, Jiang P, Wohlford-Lenane C, Lennox KA, Jacobi AM, Praekh K, Rose SD, Behlke MA, Xing Y, Welsh MJ, McCray PB Jr.

Am J Respir Cell Mol Biol. 2013 Oct;49(4):544-51. doi: 10.1165/rcmb.2012-0430OC.

49.

CFTR-deficient pigs display peripheral nervous system defects at birth.

Reznikov LR, Dong Q, Chen JH, Moninger TO, Park JM, Zhang Y, Du J, Hildebrand MS, Smith RJ, Randak CO, Stoltz DA, Welsh MJ.

Proc Natl Acad Sci U S A. 2013 Feb 19;110(8):3083-8. doi: 10.1073/pnas.1222729110. Epub 2013 Feb 4.

50.

Fear and panic in humans with bilateral amygdala damage.

Feinstein JS, Buzza C, Hurlemann R, Follmer RL, Dahdaleh NS, Coryell WH, Welsh MJ, Tranel D, Wemmie JA.

Nat Neurosci. 2013 Mar;16(3):270-2. doi: 10.1038/nn.3323. Epub 2013 Feb 3.

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