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Items: 1 to 50 of 174

1.

Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function.

de Winter-de Groot KM, Janssens HM, van Uum RT, Dekkers JF, Berkers G, Vonk A, Kruisselbrink E, Oppelaar H, Vries R, Clevers H, Houwen RHJ, Escher JC, Elias SG, de Jonge HR, de Rijke YB, Tiddens HAWM, van der Ent CK, Beekman JM.

Eur Respir J. 2018 Sep 17;52(3). pii: 1702529. doi: 10.1183/13993003.02529-2017. Print 2018 Sep.

PMID:
30166324
2.

Adenylyl cyclase 6 is involved in the hyposecretory status of experimental colitis.

Romero-Calvo I, Ocón B, Gámez-Belmonte R, Hernández-Chirlaque C, de Jonge HR, Bijvelds MJ, Martínez-Augustin O, Sánchez de Medina F.

Pflugers Arch. 2018 Aug 9. doi: 10.1007/s00424-018-2187-z. [Epub ahead of print]

PMID:
30094477
3.

Molecular Basis and Differentiation-Associated Alterations of Anion Secretion in Human Duodenal Enteroid Monolayers.

Yin J, Tse CM, Avula LR, Singh V, Foulke-Abel J, de Jonge HR, Donowitz M.

Cell Mol Gastroenterol Hepatol. 2018 Feb 9;5(4):591-609. doi: 10.1016/j.jcmgh.2018.02.002. eCollection 2018.

4.

Selective inhibition of intestinal guanosine 3',5'-cyclic monophosphate signaling by small-molecule protein kinase inhibitors.

Bijvelds MJC, Tresadern G, Hellemans A, Smans K, Nieuwenhuijze NDA, Meijsen KF, Bongartz JP, Ver Donck L, de Jonge HR, Schuurkes JAJ, De Maeyer JH.

J Biol Chem. 2018 May 25;293(21):8173-8181. doi: 10.1074/jbc.RA118.002835. Epub 2018 Apr 13.

5.

Editorial overview: Respiratory: Transformational therapies for cystic fibrosis.

Sheppard DN, Bear CE, de Jonge HR.

Curr Opin Pharmacol. 2017 Jun;34:viii-xi. doi: 10.1016/j.coph.2017.11.006. No abstract available.

PMID:
29221574
6.

Cystic Fibrosis-related Liver Disease: Research Challenges and Future Perspectives.

Debray D, Narkewicz MR, Bodewes FAJA, Colombo C, Housset C, de Jonge HR, Jonker JW, Kelly DA, Ling SC, Poynard T, Sogni P, Trauner M, Witters P, Baumann U, Wilschanski M, Verkade HJ.

J Pediatr Gastroenterol Nutr. 2017 Oct;65(4):443-448. doi: 10.1097/MPG.0000000000001676.

PMID:
28753176
7.

β2-Adrenergic receptor agonists activate CFTR in intestinal organoids and subjects with cystic fibrosis.

Vijftigschild LA, Berkers G, Dekkers JF, Zomer-van Ommen DD, Matthes E, Kruisselbrink E, Vonk A, Hensen CE, Heida-Michel S, Geerdink M, Janssens HM, van de Graaf EA, Bronsveld I, de Winter-de Groot KM, Majoor CJ, Heijerman HG, de Jonge HR, Hanrahan JW, van der Ent CK, Beekman JM.

Eur Respir J. 2016 Sep;48(3):768-79. doi: 10.1183/13993003.01661-2015. Epub 2016 Jul 28.

8.

Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis.

Dekkers JF, Berkers G, Kruisselbrink E, Vonk A, de Jonge HR, Janssens HM, Bronsveld I, van de Graaf EA, Nieuwenhuis EE, Houwen RH, Vleggaar FP, Escher JC, de Rijke YB, Majoor CJ, Heijerman HG, de Winter-de Groot KM, Clevers H, van der Ent CK, Beekman JM.

Sci Transl Med. 2016 Jun 22;8(344):344ra84. doi: 10.1126/scitranslmed.aad8278.

PMID:
27334259
9.

The phospholipid flippase ATP8B1 mediates apical localization of the cystic fibrosis transmembrane regulator.

van der Mark VA, de Jonge HR, Chang JC, Ho-Mok KS, Duijst S, Vidović D, Carlon MS, Oude Elferink RP, Paulusma CC.

Biochim Biophys Acta. 2016 Sep;1863(9):2280-8. doi: 10.1016/j.bbamcr.2016.06.005. Epub 2016 Jun 11.

10.

Guanylin and uroguanylin are produced by mouse intestinal epithelial cells of columnar and secretory lineage.

Ikpa PT, Sleddens HF, Steinbrecher KA, Peppelenbosch MP, de Jonge HR, Smits R, Bijvelds MJ.

Histochem Cell Biol. 2016 Oct;146(4):445-55. doi: 10.1007/s00418-016-1453-4. Epub 2016 May 31.

11.

Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations.

Dekkers JF, Van Mourik P, Vonk AM, Kruisselbrink E, Berkers G, de Winter-de Groot KM, Janssens HM, Bronsveld I, van der Ent CK, de Jonge HR, Beekman JM.

J Cyst Fibros. 2016 Sep;15(5):568-78. doi: 10.1016/j.jcf.2016.04.007. Epub 2016 May 5.

12.

Human Enteroids/Colonoids and Intestinal Organoids Functionally Recapitulate Normal Intestinal Physiology and Pathophysiology.

Zachos NC, Kovbasnjuk O, Foulke-Abel J, In J, Blutt SE, de Jonge HR, Estes MK, Donowitz M.

J Biol Chem. 2016 Feb 19;291(8):3759-66. doi: 10.1074/jbc.R114.635995. Epub 2015 Dec 16. Review.

13.

Value of Organoids from Comparative Epithelia Models.

Schwarz JS, de Jonge HR, Forrest JN Jr.

Yale J Biol Med. 2015 Nov 24;88(4):367-74. eCollection 2015 Dec. Review.

14.

rAAV-CFTRΔR Rescues the Cystic Fibrosis Phenotype in Human Intestinal Organoids and Cystic Fibrosis Mice.

Vidović D, Carlon MS, da Cunha MF, Dekkers JF, Hollenhorst MI, Bijvelds MJ, Ramalho AS, Van den Haute C, Ferrante M, Baekelandt V, Janssens HM, De Boeck K, Sermet-Gaudelus I, de Jonge HR, Gijsbers R, Beekman JM, Edelman A, Debyser Z.

Am J Respir Crit Care Med. 2016 Feb 1;193(3):288-98. doi: 10.1164/rccm.201505-0914OC.

PMID:
26509335
15.

ANP and CNP activate CFTR expressed in Xenopus laevis oocytes by direct activation of PKA.

Stahl K, Stahl M, de Jonge HR, Forrest JN Jr.

J Recept Signal Transduct Res. 2015;35(5):493-504. doi: 10.3109/10799893.2015.1015738. Epub 2015 May 27.

PMID:
26016495
16.

Inhibition of Heat-Stable Toxin-Induced Intestinal Salt and Water Secretion by a Novel Class of Guanylyl Cyclase C Inhibitors.

Bijvelds MJ, Loos M, Bronsveld I, Hellemans A, Bongartz JP, Ver Donck L, Cox E, de Jonge HR, Schuurkes JA, De Maeyer JH.

J Infect Dis. 2015 Dec 1;212(11):1806-15. doi: 10.1093/infdis/jiv300. Epub 2015 May 21.

PMID:
25999056
17.

Compartmentalized accumulation of cAMP near complexes of multidrug resistance protein 4 (MRP4) and cystic fibrosis transmembrane conductance regulator (CFTR) contributes to drug-induced diarrhea.

Moon C, Zhang W, Ren A, Arora K, Sinha C, Yarlagadda S, Woodrooffe K, Schuetz JD, Valasani KR, de Jonge HR, Shanmukhappa SK, Shata MT, Buddington RK, Parthasarathi K, Naren AP.

J Biol Chem. 2015 May 1;290(18):11246-57. doi: 10.1074/jbc.M114.605410. Epub 2015 Mar 11.

18.

Cholic acid induces a Cftr dependent biliary secretion and liver growth response in mice.

Bodewes FA, Bijvelds MJ, de Vries W, Baller JF, Gouw AS, de Jonge HR, Verkade HJ.

PLoS One. 2015 Feb 13;10(2):e0117599. doi: 10.1371/journal.pone.0117599. eCollection 2015.

19.

Human enteroids: preclinical models of non-inflammatory diarrhea.

Kovbasnjuk O, Zachos NC, In J, Foulke-Abel J, Ettayebi K, Hyser JM, Broughman JR, Zeng XL, Middendorp S, de Jonge HR, Estes MK, Donowitz M.

Stem Cell Res Ther. 2013;4 Suppl 1:S3. doi: 10.1186/scrt364. Epub 2013 Dec 20. Review.

20.

Cystic fibrosis: toward personalized therapies.

Ikpa PT, Bijvelds MJ, de Jonge HR.

Int J Biochem Cell Biol. 2014 Jul;52:192-200. doi: 10.1016/j.biocel.2014.02.008. Epub 2014 Feb 20. Review.

PMID:
24561283
21.

cGMP inhibition of type 3 phosphodiesterase is the major mechanism by which C-type natriuretic peptide activates CFTR in the shark rectal gland.

De Jonge HR, Tilly BC, Hogema BM, Pfau DJ, Kelley CA, Kelley MH, Melita AM, Morris MT, Viola RM, Forrest JN Jr.

Am J Physiol Cell Physiol. 2014 Feb 15;306(4):C343-53. doi: 10.1152/ajpcell.00326.2013. Epub 2013 Nov 20.

22.

Bioelectric characterization of epithelia from neonatal CFTR knockout ferrets.

Fisher JT, Tyler SR, Zhang Y, Lee BJ, Liu X, Sun X, Sui H, Liang B, Luo M, Xie W, Yi Y, Zhou W, Song Y, Keiser N, Wang K, de Jonge HR, Engelhardt JF.

Am J Respir Cell Mol Biol. 2013 Nov;49(5):837-44. doi: 10.1165/rcmb.2012-0433OC.

23.

A functional CFTR assay using primary cystic fibrosis intestinal organoids.

Dekkers JF, Wiegerinck CL, de Jonge HR, Bronsveld I, Janssens HM, de Winter-de Groot KM, Brandsma AM, de Jong NW, Bijvelds MJ, Scholte BJ, Nieuwenhuis EE, van den Brink S, Clevers H, van der Ent CK, Middendorp S, Beekman JM.

Nat Med. 2013 Jul;19(7):939-45. doi: 10.1038/nm.3201. Epub 2013 Jun 2.

PMID:
23727931
24.

Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitors.

Wilke M, Bot A, Jorna H, Scholte BJ, de Jonge HR.

PLoS One. 2012;7(12):e52070. doi: 10.1371/journal.pone.0052070. Epub 2012 Dec 21.

25.

Bile acid handling in cystic fibrosis: marked phenotypic differences between mouse models.

Bijvelds MJ, de Jonge HR, Verkade HJ.

Gastroenterology. 2012 Dec;143(6):e19-20; author reply e20. doi: 10.1053/j.gastro.2012.08.052. Epub 2012 Oct 19. No abstract available.

PMID:
23085355
26.

The small airways accordion: concurrent or alternating fluid absorption and secretion?

de Jonge HR, Sheppard DN.

J Physiol. 2012 Aug 1;590(15):3409-10. doi: 10.1113/jphysiol.2012.239657. No abstract available.

27.

Rescue of epithelial HCO3- secretion in murine intestine by apical membrane expression of the cystic fibrosis transmembrane conductance regulator mutant F508del.

Xiao F, Li J, Singh AK, Riederer B, Wang J, Sultan A, Park H, Lee MG, Lamprecht G, Scholte BJ, De Jonge HR, Seidler U.

J Physiol. 2012 Nov 1;590(21):5317-34. doi: 10.1113/jphysiol.2012.232124. Epub 2012 Jul 16.

28.

Ursodeoxycholate modulates bile flow and bile salt pool independently from the cystic fibrosis transmembrane regulator (Cftr) in mice.

Bodewes FA, Wouthuyzen-Bakker M, Bijvelds MJ, Havinga R, de Jonge HR, Verkade HJ.

Am J Physiol Gastrointest Liver Physiol. 2012 May 1;302(9):G1035-42. doi: 10.1152/ajpgi.00258.2011. Epub 2012 Feb 2.

29.

Effect of antibiotic treatment on fat absorption in mice with cystic fibrosis.

Wouthuyzen-Bakker M, Bijvelds MJ, de Jonge HR, De Lisle RC, Burgerhof JG, Verkade HJ.

Pediatr Res. 2012 Jan;71(1):4-12. doi: 10.1038/pr.2011.4.

30.

The role of rectal chloride secretion in childhood constipation.

Bekkali N, de Jonge HR, van den Wijngaard RM, van der Steeg AF, Bijlsma PB, Taminiau JA, Desjeux JF, Benninga MA.

Neurogastroenterol Motil. 2011 Nov;23(11):1007-12. doi: 10.1111/j.1365-2982.2011.01751.x. Epub 2011 Aug 17.

PMID:
21848627
31.

New clinical diagnostic procedures for cystic fibrosis in Europe.

De Boeck K, Derichs N, Fajac I, de Jonge HR, Bronsveld I, Sermet I, Vermeulen F, Sheppard DN, Cuppens H, Hug M, Melotti P, Middleton PG, Wilschanski M; ECFS Diagnostic Network Working Group; EuroCareCF WP3 Group on CF diagnosis.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S53-66. doi: 10.1016/S1569-1993(11)60009-X. Review.

32.

Mouse models of cystic fibrosis: phenotypic analysis and research applications.

Wilke M, Buijs-Offerman RM, Aarbiou J, Colledge WH, Sheppard DN, Touqui L, Bot A, Jorna H, de Jonge HR, Scholte BJ.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S152-71. doi: 10.1016/S1569-1993(11)60020-9. Review.

33.

Assessment of CFTR function in homozygous R117H-7T subjects.

de Nooijer RA, Nobel JM, Arets HG, Bot AG, van Berkhout FT, de Rijke YB, de Jonge HR, Bronsveld I.

J Cyst Fibros. 2011 Sep;10(5):326-32. doi: 10.1016/j.jcf.2011.03.009. Epub 2011 Apr 19.

34.

NHERF2 is necessary for basal activity, second messenger inhibition, and LPA stimulation of NHE3 in mouse distal ileum.

Murtazina R, Kovbasnjuk O, Chen TE, Zachos NC, Chen Y, Kocinsky HS, Hogema BM, Seidler U, de Jonge HR, Donowitz M.

Am J Physiol Cell Physiol. 2011 Jul;301(1):C126-36. doi: 10.1152/ajpcell.00311.2010. Epub 2011 Mar 23.

35.

Alterations in the proteome of the NHERF2 knockout mouse jejunal brush border membrane vesicles.

Donowitz M, Singh S, Singh P, Chakraborty M, Chen Y, Murtazina R, Gucek M, Cole RN, Zachos NC, Salahuddin FF, Kovbasnjuk O, Broere N, Smalley-Freed WG, Reynolds AB, Hubbard AL, Seidler U, Weinman E, de Jonge HR, Hogema BM, Li X.

Physiol Genomics. 2011 Jun 15;43(11):674-84. doi: 10.1152/physiolgenomics.00258.2010. Epub 2011 Mar 22.

36.

D-glucose acts via sodium/glucose cotransporter 1 to increase NHE3 in mouse jejunal brush border by a Na+/H+ exchange regulatory factor 2-dependent process.

Lin R, Murtazina R, Cha B, Chakraborty M, Sarker R, Chen TE, Lin Z, Hogema BM, de Jonge HR, Seidler U, Turner JR, Li X, Kovbasnjuk O, Donowitz M.

Gastroenterology. 2011 Feb;140(2):560-71. doi: 10.1053/j.gastro.2010.10.042. Epub 2010 Oct 23.

37.

Alterations in the proteome of the NHERF1 knockout mouse jejunal brush border membrane vesicles.

Donowitz M, Singh S, Singh P, Salahuddin FF, Chen Y, Chakraborty M, Murtazina R, Gucek M, Cole RN, Zachos NC, Kovbasnjuk O, Broere N, Smalley-Freed WG, Reynolds AB, Hubbard AL, Seidler U, Weinman E, de Jonge HR, Hogema BM, Li X.

Physiol Genomics. 2010 Nov 15;42A(3):200-10. doi: 10.1152/physiolgenomics.00001.2010. Epub 2010 Aug 24.

38.

Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine.

Robert R, Carlile GW, Liao J, Balghi H, Lesimple P, Liu N, Kus B, Rotin D, Wilke M, de Jonge HR, Scholte BJ, Thomas DY, Hanrahan JW.

Mol Pharmacol. 2010 Jun;77(6):922-30. doi: 10.1124/mol.109.062679. Epub 2010 Mar 3.

39.

Lysophosphatidic acid stimulates the intestinal brush border Na(+)/H(+) exchanger 3 and fluid absorption via LPA(5) and NHERF2.

Lin S, Yeruva S, He P, Singh AK, Zhang H, Chen M, Lamprecht G, de Jonge HR, Tse M, Donowitz M, Hogema BM, Chun J, Seidler U, Yun CC.

Gastroenterology. 2010 Feb;138(2):649-58. doi: 10.1053/j.gastro.2009.09.055. Epub 2009 Oct 1.

40.

Activation of intestinal Cl- secretion by lubiprostone requires the cystic fibrosis transmembrane conductance regulator.

Bijvelds MJ, Bot AG, Escher JC, De Jonge HR.

Gastroenterology. 2009 Sep;137(3):976-85. doi: 10.1053/j.gastro.2009.05.037. Epub 2009 May 18.

PMID:
19454284
41.

Differential roles of NHERF1, NHERF2, and PDZK1 in regulating CFTR-mediated intestinal anion secretion in mice.

Singh AK, Riederer B, Krabbenhöft A, Rausch B, Bonhagen J, Lehmann U, de Jonge HR, Donowitz M, Yun C, Weinman EJ, Kocher O, Hogema BM, Seidler U.

J Clin Invest. 2009 Mar;119(3):540-50. doi: 10.1172/JCI35541. Epub 2009 Feb 16.

42.

Defective jejunal and colonic salt absorption and alteredNa(+)/H (+) exchanger 3 (NHE3) activity in NHE regulatory factor 1 (NHERF1) adaptor protein-deficient mice.

Broere N, Chen M, Cinar A, Singh AK, Hillesheim J, Riederer B, Lünnemann M, Rottinghaus I, Krabbenhöft A, Engelhardt R, Rausch B, Weinman EJ, Donowitz M, Hubbard A, Kocher O, de Jonge HR, Hogema BM, Seidler U.

Pflugers Arch. 2009 Mar;457(5):1079-91. doi: 10.1007/s00424-008-0579-1. Epub 2008 Aug 29.

43.

Cell volume regulation in intestinal epithelial cells.

Tomassen S, de Jonge HR, Tilly BC.

Adv Exp Med Biol. 2004;559:339-47. Review. No abstract available.

PMID:
18727253
44.

Very mild disease phenotype of congenic CftrTgH(neoim)Hgu cystic fibrosis mice.

Tóth B, Wilke M, Stanke F, Dorsch M, Jansen S, Wedekind D, Charizopoulou N, Bot A, Burmester M, Leonhard-Marek S, de Jonge HR, Hedrich HJ, Breves G, Tümmler B.

BMC Genet. 2008 Apr 9;9:28. doi: 10.1186/1471-2156-9-28.

45.

Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.

Noël S, Wilke M, Bot AG, De Jonge HR, Becq F.

J Pharmacol Exp Ther. 2008 Jun;325(3):1016-23. doi: 10.1124/jpet.107.135582. Epub 2008 Feb 28.

46.

Urine electrolyte, mineral, and protein excretion in NHERF-2 and NHERF-1 null mice.

Cunningham R, Esmaili A, Brown E, Biswas RS, Murtazina R, Donowitz M, Dijkman HB, van der Vlag J, Hogema BM, De Jonge HR, Shenolikar S, Wade JB, Weinman EJ.

Am J Physiol Renal Physiol. 2008 Apr;294(4):F1001-7. doi: 10.1152/ajprenal.00504.2007. Epub 2008 Feb 6.

47.

Cystic fibrosis transmembrane conductance regulator activation is reduced in the small intestine of Na+/H+ exchanger 3 regulatory factor 1 (NHERF-1)- but Not NHERF-2-deficient mice.

Broere N, Hillesheim J, Tuo B, Jorna H, Houtsmuller AB, Shenolikar S, Weinman EJ, Donowitz M, Seidler U, de Jonge HR, Hogema BM.

J Biol Chem. 2007 Dec 28;282(52):37575-84. Epub 2007 Oct 18.

48.

Osmosignaling and volume regulation in intestinal epithelial cells.

Lim CH, Bot AG, de Jonge HR, Tilly BC.

Methods Enzymol. 2007;428:325-42. Review.

PMID:
17875427
49.

Cholesterol depletion and genistein as tools to promote F508delCFTR retention at the plasma membrane.

Lim CH, Bijvelds MJ, Nigg A, Schoonderwoerd K, Houtsmuller AB, de Jonge HR, Tilly BC.

Cell Physiol Biochem. 2007;20(5):473-82.

50.

Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis.

Poschet JF, Timmins GS, Taylor-Cousar JL, Ornatowski W, Fazio J, Perkett E, Wilson KR, Yu HD, de Jonge HR, Deretic V.

Am J Physiol Lung Cell Mol Physiol. 2007 Sep;293(3):L712-9. Epub 2007 Jun 22.

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