Format
Sort by
Items per page

Send to

Choose Destination

Search results

Items: 1 to 50 of 53

1.

Mucus strands from submucosal glands initiate mucociliary transport of large particles.

Fischer AJ, Pino-Argumedo MI, Hilkin BM, Shanrock CR, Gansemer ND, Chaly AL, Zarei K, Allen PD, Ostedgaard LS, Hoffman EA, Stoltz DA, Welsh MJ, Abou Alaiwa MH.

JCI Insight. 2019 Jan 10;4(1). pii: 124863. doi: 10.1172/jci.insight.124863. [Epub ahead of print]

2.

Widespread airway distribution and short-term phenotypic correction of cystic fibrosis pigs following aerosol delivery of piggyBac/adenovirus.

Cooney AL, Singh BK, Loza LM, Thornell IM, Hippee CE, Powers LS, Ostedgaard LS, Meyerholz DK, Wohlford-Lenane C, Stoltz DA, B McCray P Jr, Sinn PL.

Nucleic Acids Res. 2018 Oct 12;46(18):9591-9600. doi: 10.1093/nar/gky773.

3.

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.

4.

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.

5.

Alteration of protein function by a silent polymorphism linked to tRNA abundance.

Kirchner S, Cai Z, Rauscher R, Kastelic N, Anding M, Czech A, Kleizen B, Ostedgaard LS, Braakman I, Sheppard DN, Ignatova Z.

PLoS Biol. 2017 May 16;15(5):e2000779. doi: 10.1371/journal.pbio.2000779. eCollection 2017 May.

6.

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. doi: 10.1172/jci.insight.88728.

7.

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.

8.

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.

9.

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.

10.

Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility.

Cook DP, Rector MV, Bouzek DC, Michalski AS, Gansemer ND, Reznikov LR, Li X, Stroik MR, Ostedgaard LS, Abou Alaiwa MH, Thompson MA, Prakash YS, Krishnan R, Meyerholz DK, Seow CY, Stoltz DA.

Am J Respir Crit Care Med. 2016 Feb 15;193(4):417-26. doi: 10.1164/rccm.201508-1562OC.

12.

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.

13.

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.

14.

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.

15.

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.

16.

A microRNA network regulates expression and biosynthesis of wild-type and DeltaF508 mutant cystic fibrosis transmembrane conductance regulator.

Ramachandran S, Karp PH, Jiang P, Ostedgaard LS, Walz AE, Fisher JT, Keshavjee S, Lennox KA, Jacobi AM, Rose SD, Behlke MA, Welsh MJ, Xing Y, McCray PB Jr.

Proc Natl Acad Sci U S A. 2012 Aug 14;109(33):13362-7. doi: 10.1073/pnas.1210906109. Epub 2012 Aug 1.

17.

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.

18.

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.

19.

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.

20.

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.

21.

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.

22.

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.

23.

Processing and function of CFTR-DeltaF508 are species-dependent.

Ostedgaard LS, Rogers CS, Dong Q, Randak CO, Vermeer DW, Rokhlina T, Karp PH, Welsh MJ.

Proc Natl Acad Sci U S A. 2007 Sep 25;104(39):15370-5. Epub 2007 Sep 14.

24.

A shortened adeno-associated virus expression cassette for CFTR gene transfer to cystic fibrosis airway epithelia.

Ostedgaard LS, Rokhlina T, Karp PH, Lashmit P, Afione S, Schmidt M, Zabner J, Stinski MF, Chiorini JA, Welsh MJ.

Proc Natl Acad Sci U S A. 2005 Feb 22;102(8):2952-7. Epub 2005 Feb 9.

25.

Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

Berger AL, Randak CO, Ostedgaard LS, Karp PH, Vermeer DW, Welsh MJ.

J Biol Chem. 2005 Feb 18;280(7):5221-6. Epub 2004 Dec 6.

26.

Methods to study CFTR protein in vitro.

Benos DJ, Berdiev BK, Ismailov II, Ostedgaard LS, Kogan I, Li C, Ramjeesingh M, Bear CE.

J Cyst Fibros. 2004 Aug;3 Suppl 2:79-83. Review.

27.

Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.

Ostedgaard LS, Randak C, Rokhlina T, Karp P, Vermeer D, Ashbourne Excoffon KJ, Welsh MJ.

Proc Natl Acad Sci U S A. 2003 Feb 18;100(4):1937-42. Epub 2003 Feb 10.

28.

CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo.

Ostedgaard LS, Zabner J, Vermeer DW, Rokhlina T, Karp PH, Stecenko AA, Randak C, Welsh MJ.

Proc Natl Acad Sci U S A. 2002 Mar 5;99(5):3093-8. Epub 2002 Feb 19.

29.

Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by its R domain.

Ostedgaard LS, Baldursson O, Welsh MJ.

J Biol Chem. 2001 Mar 16;276(11):7689-92. Epub 2001 Jan 23. Review. No abstract available.

30.

Cystic fibrosis transmembrane conductance regulator Cl- channels with R domain deletions and translocations show phosphorylation-dependent and -independent activity.

Baldursson O, Ostedgaard LS, Rokhlina T, Cotten JF, Welsh MJ.

J Biol Chem. 2001 Jan 19;276(3):1904-10. Epub 2000 Oct 18.

31.

A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution.

Ostedgaard LS, Baldursson O, Vermeer DW, Welsh MJ, Robertson AD.

Proc Natl Acad Sci U S A. 2000 May 9;97(10):5657-62.

32.

Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR.

Ostedgaard LS, Zeiher B, Welsh MJ.

J Cell Sci. 1999 Jul;112 ( Pt 13):2091-8.

33.

Structural biology. The ABC of a versatile engine.

Welsh MJ, Robertson AD, Ostedgaard LS.

Nature. 1998 Dec 17;396(6712):623-4. No abstract available.

PMID:
9872305
34.
35.

Cystic fibrosis problem probed by proteolysis.

Welsh MJ, Ostedgaard LS.

Nat Struct Biol. 1998 Mar;5(3):167-9. No abstract available.

PMID:
9501902
36.

Association of domains within the cystic fibrosis transmembrane conductance regulator.

Ostedgaard LS, Rich DP, DeBerg LG, Welsh MJ.

Biochemistry. 1997 Feb 11;36(6):1287-94.

PMID:
9063876
37.
38.

Understanding how cystic fibrosis mutations cause a loss of Cl- channel function.

Sheppard DN, Ostedgaard LS.

Mol Med Today. 1996 Jul;2(7):290-7. Review.

PMID:
8796909
40.

Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium.

Sheppard DN, Carson MR, Ostedgaard LS, Denning GM, Welsh MJ.

Am J Physiol. 1994 Apr;266(4 Pt 1):L405-13.

PMID:
7513963
41.

The amino-terminal portion of CFTR forms a regulated Cl- channel.

Sheppard DN, Ostedgaard LS, Rich DP, Welsh MJ.

Cell. 1994 Mar 25;76(6):1091-8.

PMID:
7511062
42.

Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro.

Marshall J, Fang S, Ostedgaard LS, O'Riordan CR, Ferrara D, Amara JF, Hoppe H 4th, Scheule RK, Welsh MJ, Smith AE, et al.

J Biol Chem. 1994 Jan 28;269(4):2987-95.

43.

Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast.

Teem JL, Berger HA, Ostedgaard LS, Rich DP, Tsui LC, Welsh MJ.

Cell. 1993 Apr 23;73(2):335-46.

PMID:
7682896
44.

Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.

Sheppard DN, Rich DP, Ostedgaard LS, Gregory RJ, Smith AE, Welsh MJ.

Nature. 1993 Mar 11;362(6416):160-4.

PMID:
7680769
45.

Dysfunction of CFTR bearing the delta F508 mutation.

Welsh MJ, Denning GM, Ostedgaard LS, Anderson MP.

J Cell Sci Suppl. 1993;17:235-9. Review.

PMID:
7511616
46.

Partial purification of the cystic fibrosis transmembrane conductance regulator.

Ostedgaard LS, Welsh MJ.

J Biol Chem. 1992 Dec 25;267(36):26142-9.

48.

Staphylococcus aureus alpha-toxin permeabilizes the basolateral membrane of a Cl(-)-secreting epithelium.

Ostedgaard LS, Shasby DM, Welsh MJ.

Am J Physiol. 1992 Jul;263(1 Pt 1):L104-12.

PMID:
1322049
49.

Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers.

Tilly BC, Winter MC, Ostedgaard LS, O'Riordan C, Smith AE, Welsh MJ.

J Biol Chem. 1992 May 15;267(14):9470-3.

50.

Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation.

Welsh MJ, Anderson MP, Rich DP, Berger HA, Denning GM, Ostedgaard LS, Sheppard DN, Cheng SH, Gregory RJ, Smith AE.

Neuron. 1992 May;8(5):821-9. Review. No abstract available.

PMID:
1375035

Supplemental Content

Loading ...
Support Center