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Publications

PubMed Archive 

Selected Publications 

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.
PMID: 26823428

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.
PMID: 26808501

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.
PMID: 26180203

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

Mutating the Conserved Q-loop Glutamine 1291 Selectively Disrupts Adenylate Kinase-dependent Channel Gating of the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and Reduces Channel Function in Primary Human Airway Epithelia.
Dong Q, Ernst SE, Ostedgaard LS, Shah VS, Ver Heul AR, Welsh MJ, Randak CO.
J Biol Chem. 2015 May 29;290(22):14140-53. doi: 10.1074/jbc.M114.611616. Epub 2015 Apr 17.
PMID: 25887396

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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, 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 3;123(6):2685-93. doi: 10.1172/JCI68867. Epub 2013 May 8.

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.

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.

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.

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.

Demonstration of phosphoryl group transfer indicates that the ATP-binding cassette (ABC) transporter cystic fibrosis transmembrane conductance regulator (CFTR) exhibits adenylate kinase activity. 
Randak CO, Ver Heul AR, Welsh MJ. J Biol Chem. 2012 Oct 19;287(43):36105-10. doi: 10.1074/jbc.M112.408450. Epub 2012 Sep 4.

Pancreatic and biliary secretion are both altered in cystic fibrosis pigs. 
Uc A, Giriyappa R, Meyerholz DK, Griffin M, Ostedgaard LS, Tang XX, Abu-El-Haija M, Stoltz DA, Ludwig P, Pezzulo A, Abu-El-Haija M, Taft P, Welsh MJ. Am J Physiol Gastrointest Liver Physiol. 2012 Oct 15;303(8):G961-8. doi: 10.1152/ajpgi.00030.2012. Epub 2012 Aug 30.

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. 
Pezzulo AA, Tang XX, Hoegger MJ, Alaiwa MH, Ramachandran S, Moninger TO, Karp PH, Wohlford-Lenane CL, Haagsman HP, van Eijk M, Bánfi B, Horswill AR, Stoltz DA, McCray PB Jr, Welsh MJ, Zabner J. Nature. 2012 Jul 4;487(7405):109-13. doi: 10.1038/nature11130.

CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia. 
Li X, Comellas AP, Karp PH, Ernst SE, Moninger TO, Gansemer ND, Taft PJ, Pezzulo AA, Rector MV, Rossen N, Stoltz DA, McCray PB Jr, Welsh MJ, Zabner J. Am J Physiol Lung Cell Mol Physiol. 2012 Jul;303(2):L152-60. doi: 10.1152/ajplung.00116.2012. Epub 2012 May 25.

Detecting activity-evoked pH changes in human brain. 
Magnotta VA, Heo HY, Dlouhy BJ, Dahdaleh NS, Follmer RL, Thedens DR, Welsh MJ, Wemmie JA. Proc Natl Acad Sci U S A. 2012 May 22;109(21):8270-3. doi: 10.1073/pnas.1205902109. Epub 2012 May 7.

ppk23-Dependent chemosensory functions contribute to courtship behavior in Drosophila melanogaster.
Lu B, LaMora A, Sun Y, Welsh MJ, Ben-Shahar Y. PLoS Genet. 2012;8(3):e1002587. doi: 10.1371/journal.pgen.1002587. Epub 2012 Mar 15.

Human-mouse cystic fibrosis transmembrane conductance regulator (CFTR) chimeras identify regions that partially rescue CFTR-ΔF508 processing and alter its gating defect.
Dong Q, Ostedgaard LS, Rogers C, Vermeer DW, Zhang Y, Welsh MJ. Proc Natl Acad Sci U S A. 2012 Jan 17;109(3):917-22. doi: 10.1073/pnas.1120065109. Epub 2011 Dec 30.

Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.
Itani OA, Chen JH, Karp PH, Ernst S, Keshavjee S, Parekh K, Klesney-Tait J, Zabner J, Welsh MJ. Proc Natl Acad Sci U S A. 2011 Jun 21;108(25):10260-5. doi: 10.1073/pnas.1106695108. Epub 2011 Jun 6.

The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. 
Ostedgaard LS, Meyerholz DK, Chen JH, Pezzulo AA, Karp PH, Rokhlina T, Ernst SE, Hanfland RA, Reznikov LR, Ludwig PS, Rogan MP, Davis GJ, Dohrn CL, Wohlford-Lenane C, Taft PJ, Rector MV, Hornick E, Nassar BS, Samuel M, Zhang Y, Richter SS, Uc A, Shilyansky J, Prather RS, McCray PB Jr, Zabner J, Welsh MJ, Stoltz DA. Sci Transl Med. 2011 Mar 16;3(74):74ra24. doi: 10.1126/scitranslmed.3001868.

Expressing acid-sensing ion channel 3 in the brain alters acid-evoked currents and impairs fear conditioning.
Vralsted VC, Price MP, Du J, Schnizler M, Wunsch AM, Ziemann AE, Welsh MJ, Wemmie JA. Genes Brain Behav. 2011 Jun;10(4):444-50. doi: 10.1111/j.1601-183X.2011.00683.x. Epub 2011 Mar 7.

Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice.
Ostedgaard LS, Meyerholz DK, Vermeer DW, Karp PH, Schneider L, Sigmund CD, Welsh MJ. Proc Natl Acad Sci U S A. 2011 Feb 15;108(7):2921-6. doi: 10.1073/pnas.1019752108. Epub 2011 Feb 1.

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. 
Chen JH, Stoltz DA, Karp PH, Ernst SE, Pezzulo AA, Moninger TO, Rector MV, Reznikov LR, Launspach JL, Chaloner K, Zabner J, Welsh MJ. Cell. 2010 Dec 10;143(6):911-23. doi: 10.1016/j.cell.2010.11.029.

Targeting the basic defect in cystic fibrosis.
Welsh MJ. N Engl J Med. 2010 Nov 18;363(21):2056-7. doi: 10.1056/NEJMe1010123.

Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
Rogan MP, Reznikov LR, Pezzulo AA, Gansemer ND, Samuel M, Prather RS, Zabner J, Fredericks DC, McCray PB Jr, Welsh MJ, Stoltz DA. Proc Natl Acad Sci U S A. 2010 Nov 23;107(47):20571-5. doi: 10.1073/pnas.1015281107. Epub 2010 Nov 8.

Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. 
Meyerholz DK, Stoltz DA, Namati E, Ramachandran S, Pezzulo AA, Smith AR, Rector MV, Suter MJ, Kao S, McLennan G, Tearney GJ, Zabner J, McCray PB Jr, Welsh MJ. Am J Respir Crit Care Med. 2010 Nov 15;182(10):1251-61. doi: 10.1164/rccm.201004-0643OC. Epub 2010 Jul 9.

Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. 
Stoltz DA, Meyerholz DK, Pezzulo AA, Ramachandran S, Rogan MP, Davis GJ, Hanfland RA, Wohlford-Lenane C, Dohrn CL, Bartlett JA, Nelson GA 4th, Chang EH, Taft PJ, Ludwig PS, Estin M, Hornick EE, Launspach JL, Samuel M, Rokhlina T, Karp PH, Ostedgaard LS, Uc A, Starner TD, Horswill AR, Brogden KA, Prather RS, Richter SS, Shilyansky J, McCray PB Jr, Zabner J, Welsh MJ. Sci Transl Med. 2010 Apr 28;2(29):29ra31. doi: 10.1126/scitranslmed.3000928.

The Drosophila gene CheB42a is a novel modifier of Deg/ENaC channel function.
Ben-Shahar Y, Lu B, Collier DM, Snyder PM, Schnizler M, Welsh MJ. PLoS One. 2010 Feb 24;5(2):e9395. doi: 10.1371/journal.pone.0009395.

Pathology of gastrointestinal organs in a porcine model of cystic fibrosis.
Meyerholz DK, Stoltz DA, Pezzulo AA, Welsh MJ. Am J Pathol. 2010 Mar;176(3):1377-89. doi: 10.2353/ajpath.2010.090849. Epub 2010 Jan 28.

The amygdala is a chemosensor that detects carbon dioxide and acidosis to elicit fear behavior.
Ziemann AE, Allen JE, Dahdaleh NS, Drebot II, Coryell MW, Wunsch AM, Lynch CM, Faraci FM, Howard MA 3rd, Welsh MJ, Wemmie JA. Cell. 2009 Nov 25;139(5):1012-21. doi: 10.1016/j.cell.2009.10.029.

Development of a porcine model of cystic fibrosis. 
Welsh MJ, Rogers CS, Stoltz DA, Meyerholz DK, Prather RS. Trans Am Clin Climatol Assoc. 2009;120:149-62.

Motile cilia of human airway epithelia are chemosensory. 
Shah AS, Ben-Shahar Y, Moninger TO, Kline JN, Welsh MJ. Science. 2009 Aug 28;325(5944):1131-4. doi: 10.1126/science.1173869. Epub 2009 Jul 23.

ASIC2 subunits target acid-sensing ion channels to the synapse via an association with PSD-95. 
Zha XM, Costa V, Harding AM, Reznikov L, Benson CJ, Welsh MJ. J Neurosci. 2009 Jul 1;29(26):8438-46. doi: 10.1523/JNEUROSCI.1284-09.2009.

Oxidant regulated inter-subunit disulfide bond formation between ASIC1a subunits.
Zha XM, Wang R, Collier DM, Snyder PM, Wemmie JA, Welsh MJ. Proc Natl Acad Sci U S A. 2009 Mar 3;106(9):3573-8. doi: 10.1073/pnas.0813402106. Epub 2009 Feb 13.

Acute regulation of tight junction ion selectivity in human airway epithelia. 
Flynn AN, Itani OA, Moninger TO, Welsh MJ. Proc Natl Acad Sci U S A. 2009 Mar 3;106(9):3591-6. doi: 10.1073/pnas.0813393106. Epub 2009 Feb 10.

The piglet as a model for B cell and immune system development. 
Butler JE, Lager KM, Splichal I, Francis D, Kacskovics I, Sinkora M, Wertz N, Sun J, Zhao Y, Brown WR, DeWald R, Dierks S, Muyldermans S, Lunney JK, McCray PB, Rogers CS, Welsh MJ, Navarro P, Klobasa F, Habe F, Ramsoondar J. Vet Immunol Immunopathol. 2009 Mar 15;128(1-3):147-70. doi: 10.1016/j.vetimm.2008.10.321. Epub 2008 Nov 1. Review.

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. 
Rogers CS, Stoltz DA, Meyerholz DK, Ostedgaard LS, Rokhlina T, Taft PJ, Rogan MP, Pezzulo AA, Karp PH, Itani OA, Kabel AC, Wohlford-Lenane CL, Davis GJ, Hanfland RA, Smith TL, Samuel M, Wax D, Murphy CN, Rieke A, Whitworth K, Uc A, Starner TD, Brogden KA, Shilyansky J, McCray PB Jr, Zabner J, Prather RS, Welsh MJ. Science. 2008 Sep 26;321(5897):1837-41. doi: 10.1126/science.1163600.

A mutation in CFTR modifies the effects of the adenylate kinase inhibitor Ap5A on channel gating. 
Dong Q, Randak CO, Welsh MJ. Biophys J. 2008 Dec;95(11):5178-85. doi: 10.1529/biophysj.108.140897. Epub 2008 Sep 19.

Acid-sensing ion channel 3 (ASIC3) cell surface expression is modulated by PSD-95 within lipid rafts. 
Eshcol JO, Harding AM, Hattori T, Costa V, Welsh MJ, Benson CJ. Am J Physiol Cell Physiol. 2008 Sep;295(3):C732-9. doi: 10.1152/ajpcell.00514.2007. Epub 2008 Jun 25.

Seizure termination by acidosis depends on ASIC1a. 
Ziemann AE, Schnizler MK, Albert GW, Severson MA, Howard MA 3rd, Welsh MJ, Wemmie JA. Nat Neurosci. 2008 Jul;11(7):816-22. doi: 10.1038/nn.2132. Epub 2008 Jun 8.

The porcine lung as a potential model for cystic fibrosis. 
Rogers CS, Abraham WM, Brogden KA, Engelhardt JF, Fisher JT, McCray PB Jr, McLennan G, Meyerholz DK, Namati E, Ostedgaard LS, Prather RS, Sabater JR, Stoltz DA, Zabner J, Welsh MJ. Am J Physiol Lung Cell Mol Physiol. 2008 Aug;295(2):L240-63. doi: 10.1152/ajplung.90203.2008. Epub 2008 May 16. Review.