Cystic fibrosis transmembrane conductance regulator knockout mice exhibit aberrant gastrointestinal microbiota
Open accessAbstract
The composition of the gastrointestinal microbiome is increasingly recognized as a crucial contributor to immune and metabolic homeostasis-deficiencies in which are characteristic of cystic fibrosis (CF) patients. The murine model (CFTR (-/-) , CF), has, in previous studies, demonstrated characteristic CF gastrointestinal (GI) manifestations including slowed transit and significant upregulation of genes associated with inflammation. To determine if characteristics of the microbiome are associated with these phenotypes we used a phylogenetic microarray to compare small intestine bacterial communities of wild type and congenic CF mice. Loss of functional CFTR is associated with significant decreases in GI bacterial community richness, evenness and diversity and reduced relative abundance of putative protective species such as Acinetobacter lwoffii and a multitude of Lactobacilliales members. CF mice exhibited significant enrichment of Mycobacteria species and Bacteroides fragilis, previously associated with GI infection and immunomodulation. Antibiotic administration to WT and CF animals resulted in convergence of their microbiome composition and significant increases in community diversity in CF mice. These communities were characterized by enrichment of members of the Lactobacillaceae and Bifidobacteriaceae and reduced abundance of Enterobacteriaceae and Clostridiaceae. These data suggest that Enterobacteria and Clostridia species, long associated with small intestinal overgrowth and inflammatory bowel disease, may suppress both ileal bacterial diversity and the particular species which maintain motility and immune homeostasis in this niche. Thus, these data provide the first indications that GI bacterial colonization is strongly impacted by the loss of functional CFTR and opens up avenues for alternative therapeutic approaches to improve CF disease management.
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Evidence suggests that in cystic fibrosis (CF) patients the lung disease determines the vital prognosis.
“Evidence suggests that in CF patients the lung disease determines the vital prognosis [85] .”
From Intestinal microbiota in health and disease: Role of bifidobacteria in gut homeostasis (2014) · cites this paper as [85]
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vegan: Community Ecology Package
Oksanen, J., Simpson, G. L., Blanchet, F. G., Kindt, R., Legendre, P., Minchin, P. R., O’Hara, R. B., Sólymos, P., Stevens, M. H. H., Szöcs, E., et al. (2001) · doi:10.32614/cran.package.vegan · Cited by 27,099 · see citing works
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418 · see citing works
Induction of Intestinal Th17 Cells by Segmented Filamentous Bacteria
Ivanov, I. I., Atarashi, K., Manel, N., Brodie, E. L., Shima, T., Karaoz, U., Wei, D., Goldfarb, K. C., Santee, C. A., Lynch, S. V., et al. (2009) · Cell · doi:10.1016/j.cell.2009.09.033 · Cited by 4,554 · see citing works
Self-citationIdentification of the Cystic Fibrosis Gene: Genetic Analysis
Kerem, B.-S., Rommens, J. M., Buchanan, J. A., Markiewicz, D., Cox, T. K., Chakravarti, A., Buchwald, M., & Tsui, L.-C. (1989) · Science · doi:10.1126/science.2570460 · Cited by 4,142 · see citing works
Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping
Rommens, J. M., Iannuzzi, M. C., Kerem, B.-S., Drumm, M. L., Melmer, G., Dean, M. C., Rozmahel, R. F., Cole, J. L., Kennedy, D., Hidaka, N., et al. (1989) · Science · doi:10.1126/science.2772657 · Cited by 3,238 · see citing works
Dethlefsen, L., Huse, S., Sogin, M. L., & Relman, D. A. (2008) · PLoS Biology · doi:10.1371/journal.pbio.0060280 · Cited by 2,361 · see citing works
Factors influencing the composition of the intestinal microbiota in early infancy
Penders, J., Thijs, C., Vink, C., Stelma, F. F., Snijders, B., Kummeling, I., van den Brandt, P. A., & Stobberingh, E. E. (2006) · PEDIATRICS · doi:10.1542/peds.2005-2824 · Cited by 2,232 · see citing works
Diet Drives Convergence in Gut Microbiome Functions Across Mammalian Phylogeny and Within Humans
Muegge, B. D., Kuczynski, J., Knights, D., Clemente, J. C., González, A., Fontana, L., Henrissat, B., Knight, R., & Gordon, J. I. (2011) · Science · doi:10.1126/science.1198719 · Cited by 1,988 · see citing works
The Toll-like receptor 2 pathway establishes colonization by a commensal of the human microbiota
Round, J. L., Lee, S. M., Li, J., Tran, G., Jabri, B., Chatila, T. A., & Mazmanian, S. K. (2011) · Science · doi:10.1126/science.1206095 · Cited by 1,604 · see citing works
Gut inflammation provides a respiratory electron acceptor for Salmonella
Winter, S., Thiennimitr, P., Winter, M. G., Butler, B. P., Huseby, D. L., Crawford, R. W., Russell, J. M., Bevins, C. L., Adams, L. G., Tsolis, R. M., et al. (2010) · Nature · doi:10.1038/nature09415 · Cited by 1,271 · see citing works
Hajishengallis, G., Liang, S., Payne, M. A., Hashim, A., Jotwani, R., Eskan, M. A., McIntosh, M. L., Alsam, A., Kirkwood, K. L., Lambris, J. D., et al. (2011) · Cell Host & Microbe · doi:10.1016/j.chom.2011.10.006 · Cited by 1,154 · see citing works
Penders, J., Thijs, C., van den Brandt, P. A., Kummeling, I., Snijders, B., Stelma, F., Adams, H., van Ree, R., & Stobberingh, E. E. (2007) · Gut · doi:10.1136/gut.2006.100164 · Cited by 844 · see citing works
Implication of the Mosquito Midgut Microbiota in the Defense against Malaria Parasites
Dong, Y., Manfredini, F., & Dimopoulos, G. (2009) · PLoS Pathogens · doi:10.1371/journal.ppat.1000423 · Cited by 806 · see citing works
Sonnenburg, E. D., Zheng, H., Joglekar, P., Higginbottom, S. K., Firbank, S. J., Bolam, D. N., & Sonnenburg, J. L. (2010) · Cell · doi:10.1016/j.cell.2010.05.005 · Cited by 745 · see citing works
Urban aerosols harbor diverse and dynamic bacterial populations
Brodie, E., DeSantis, T. Z., Parker, J. M., Zubieta, I. X., Piceno, Y. M., & Andersen, G. L. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0608255104 · Cited by 642 · see citing works
Self-citationKwon, H., Lee, C., So, J., Chae, C.-S., Hwang, J.-S., Sahoo, A., Nam, J. H., Rhee, J. H., Hwang, K.-C., & Im, S. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0904055107 · Cited by 627 · see citing works
DeSantis, T. Z., Brodie, E., Parker, J. M., Zubieta, I. X., Piceno, Y. M., & Andersen, G. L. (2007) · Microbial Ecology · doi:10.1007/s00248-006-9134-9 · Cited by 509 · see citing works
Self-citationBrodie, E., DeSantis, T. Z., Joyner, D. C., Baek, S. M., Larsen, J. T., Andersen, G. L., Hazen, T. C., Richardson, P. M., Herman, D. J., Tokunaga, T. K., et al. (2006) · Applied and Environmental Microbiology · doi:10.1128/aem.00246-06 · Cited by 437 · see citing works
Self-citationCandela, M., Perna, F., Carnevali, P., Vitali, B., Ciati, R., Gionchetti, P., Rizzello, F., Campieri, M., & Brigidi, P. (2008) · International Journal of Food Microbiology · doi:10.1016/j.ijfoodmicro.2008.04.012 · Cited by 400 · see citing works
Penders, J., Vink, C., Driessen, C., London, N. H. H. J., Thijs, C. T., & Stobberingh, E. E. (2004) · FEMS Microbiology Letters · doi:10.1016/j.femsle.2004.11.052 · Cited by 380 · see citing works
Lawley, T. D., Clare, S., Walker, A. W., Goulding, D., Stabler, R. A., Croucher, N., Mastroeni, P., Scott, P., Raisen, C., Mottram, L., et al. (2009) · Infection and Immunity · doi:10.1128/iai.00558-09 · Cited by 349 · see citing works
Conrad, M. L., Ferstl, R., Teich, R., Brand, S., Blümer, N., Yildirim, A. Ö., Patrascan, C. C., Hanuszkiewicz, A., Akira, S., Wagner, H., et al. (2009) · The Journal of Experimental Medicine · doi:10.1084/jem.20090845 · Cited by 317 · see citing works
Brand, S., Teich, R., Dicke, T., Harb, H., Yildirim, A. Ö., Tost, J., Schneider‐Stock, R., Waterland, R. A., Bauer, U., von Mutius, E., et al. (2011) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2011.04.035 · Cited by 176 · see citing works
Norkina, O., Burnett, T. G., & Lisle, R. C. D. (2004) · Infection and Immunity · doi:10.1128/iai.72.10.6040-6049.2004 · Cited by 175 · see citing works
Escherichia coli Gene Expression Responsive to Levels of the Response Regulator EvgA
Masuda, N., & Church, G. M. (2002) · Journal of Bacteriology · doi:10.1128/jb.184.22.6225-6234.2002 · Cited by 173 · see citing works
Inflammation of the cystic fibrosis mouse small intestine
Norkina, O., Kaur, S., Ziemer, D. M., & Lisle, R. C. D. (2004) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00473.2003 · Cited by 157 · see citing works
Andréjak, C., Lescure, F. X., Pukenytė, E., Douadi, Y., Yazdanpanah, Y., Laurans, G., Schmit, J. L., Jounieaux, V., & and the Xenopi Group (2008) · Thorax · doi:10.1136/thx.2008.096842 · Cited by 99 · see citing works
Lisle, R. C. D., Roach, E., & Jansson, K. P. (2007) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00195.2007 · Cited by 89 · see citing works
Small Intestinal Mucosal Immunity and Morphometry in Luminal Overgrowth of Indigenous Gut Flora
Riordan, S. M., McIver, C. J., Wakefield, D., Duncombe, V. M., Thomas, M. C., & Bolin, T. D. (2001) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2001.03533.x · Cited by 88 · see citing works
Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine
Lisle, R. C. D. (2007) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00548.2006 · Cited by 85 · see citing works
Smith, D., Lindholm-Levy, P. J., Huitt, G. A., Heifets, L. B., & Cook, J. L. (2000) · Clinical Infectious Diseases · doi:10.1086/313693 · Cited by 81 · see citing works
Dietary changes improve survival of CFTR S489X homozygous mutant mouse
Eckman, E. A., Cotton, C. U., Kube, D. M., & Davis, P. B. (1995) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.1995.269.5.l625 · Cited by 80 · see citing works
Marcinkiewicz, J., Ciszek‐Lenda, M., Bobek, M., Strus, M., Heczko, P., Kurnyta, M., Biedroń, R., & Chmielarczyk, A. (2007) · International Journal of Experimental Pathology · doi:10.1111/j.1365-2613.2007.00530.x · Cited by 57 · see citing works
Changes in strategies for optimal antibacterial therapy in cystic fibrosis
Ratjen, F. A. (2001) · International Journal of Antimicrobial Agents · doi:10.1016/s0924-8579(00)00333-2 · Cited by 48 · see citing works
Yoshida, Y., Tsukahara, T., & Ushida, K. (2009) · Animal Science Journal · doi:10.1111/j.1740-0929.2009.00692.x · Cited by 45 · see citing works
Intestinal microbiota in short bowel syndrome
Goulet, O., & Joly, F. (2010) · Gastroentérologie Clinique et Biologique · doi:10.1016/s0399-8320(10)70019-1 · Cited by 31 · see citing works
Dahlqvist, G., & Piessevaux, H. H. (2011) · PubMed · Cited by 30 · see citing works
Prevalence of Enterotoxigenic Bacteroides fragilis in patients with diarrhea: A controlled study
Durmaz, B., Dalgalar, M., & Durmaz, R. (2005) · Anaerobe · doi:10.1016/j.anaerobe.2005.06.001 · Cited by 27 · see citing works
Sonnenburg, J. L. (2010) · Nature · doi:10.1038/464837a · Cited by 20 · see citing works
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- Penders J, Thijs C, van den Brandt PA, Kummeling I, Snijders B, Stelma F, et al. Gut microbiota composition and development of atopic manifestations in infancy: the KOALA Birth Cohort Study. Gut 2007; 56:661- 7; PMID:17047098; http://dx.doi.org/10.1136/ gut.2006.100164. · DOI · Paper page
Cited by
Sorted by citations
Role of the gut microbiota in immunity and inflammatory disease
Kamada, N., Seo, S.-U., Chen, G. Y., & Núñez, G. (2013) · Nature reviews. Immunology · doi:10.1038/nri3430 · Cited by 2,256 · see citing works
Impacts of polystyrene microplastic on the gut barrier, microbiota and metabolism of mice
Jin, Y., Lu, L., Tu, W., Luo, T., & Fu, Z. (2019) · Science of The Total Environment · doi:10.1016/j.scitotenv.2018.08.353 · Cited by 1,100 · see citing works
Intestinal microbiota in health and disease: Role of bifidobacteria in gut homeostasis
Tojo, R. (2014) · World Journal of Gastroenterology · doi:10.3748/wjg.v20.i41.15163 · Cited by 533 · see citing works
Heterogeneity of the gut microbiome in mice: guidelines for optimizing experimental design
Laukens, D., Brinkman, B. M. N., Raes, J., Vos, M. D., & Vandenabeele, P. (2015) · FEMS Microbiology Reviews · doi:10.1093/femsre/fuv036 · Cited by 378 · see citing works
Host Genetics and Gut Microbiome: Challenges and Perspectives
Kurilshikov, A., Wijmenga, C., Fu, J., & Zhernakova, A. (2017) · Trends in Immunology · doi:10.1016/j.it.2017.06.003 · Cited by 309 · see citing works
The Gut Microbiota and Respiratory Diseases: New Evidence
Li, C., Haiyue, L., Yanxia, L., Jianbing, P., & Su, J. (2020) · Journal of Immunology Research · doi:10.1155/2020/2340670 · Cited by 267 · see citing works
Tomas, J., Mulet, C., Saffarian, A., Cavin, J., Ducroc, R. A., Regnault, B., Tan, C. K., Duszka, K., Burcelin, R., Wahli, W., et al. (2016) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1612559113 · Cited by 255 · see citing works
Role of ion channels in gastrointestinal cancer
Anderson, K. J., Cormier, R. T., & Scott, P. M. (2019) · World Journal of Gastroenterology · doi:10.3748/wjg.v25.i38.5732 · Cited by 204 · see citing works
Schutte, A. H., Ermund, A., Becker‐Pauly, C., Johansson, M., Rodríguez-Piñeiro, A. M., Bäckhed, F., Müller, S., Lottaz, D., Bond, J. S., & Hansson, G. C. (2014) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1407597111 · Cited by 190 · see citing works
Characterization of Microbial Dysbiosis and Metabolomic Changes in Dogs with Acute Diarrhea
Guard, B., Barr, J. W., Reddivari, L., Klemashevich, C. L., Jayaraman, A., Steiner, J., Vanamala, J. K. P., & Suchodolski, J. (2015) · PLoS ONE · doi:10.1371/journal.pone.0127259 · Cited by 169 · see citing works
The altered gut microbiota in adults with cystic fibrosis
Burke, D. G., Fouhy, F., Harrison, M. J., Rea, M. C., Cotter, P. D., O’Sullivan, O., Stanton, C., Hill, C., Shanahan, F., Plant, B. J., et al. (2017) · BMC Microbiology · doi:10.1186/s12866-017-0968-8 · Cited by 179 · see citing works
Hoen, A. G., Li, J., Moulton, L. A., O’Toole, G. A., Housman, M. L., Koestler, D. C., Guill, M. F., Moore, J. H., Hibberd, P. L., Morrison, H. G., et al. (2015) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2015.02.049 · Cited by 165 · see citing works
Jia, R., Han, J., Liu, X., Li, K., Lai, W., Bian, L., Yan, J., & Xi, Z. (2023) · Toxics · doi:10.3390/toxics11020127 · Cited by 150 · see citing works
del Campo, R., Garriga, M. J., Pérez-Aragón, A., Guallarte, P., Lamas, A., Máiz, L., Bayón, C., Roy, G., Cantón, R., Zamora, J., et al. (2014) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2014.02.007 · Cited by 137 · see citing works
Disrupted progression of the intestinal microbiota with age in children with cystic fibrosis
Nielsen, S. J., Needham, B., Leach, S. T., Day, A. S., Jaffé, A., Thomas, T., & Ooi, C. Y. (2016) · Scientific Reports · doi:10.1038/srep24857 · Cited by 120 · see citing works
Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine
Dorsey, J. M., & Gonska, T. Y. (2017) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2017.07.014 · Cited by 118 · see citing works
The Microbiome in Cystic Fibrosis Pulmonary Disease
Françoise, A., & Héry-Arnaud, G. (2020) · Genes · doi:10.3390/genes11050536 · Cited by 115 · see citing works
Interactions between the gut microbiome and host gene regulation in cystic fibrosis
Dayama, G., Priya, S., Niccum, D. E., Khoruts, A., & Blekhman, R. (2020) · Genome Medicine · doi:10.1186/s13073-020-0710-2 · Cited by 114 · see citing works
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases
Fabris, L., Fiorotto, R., Spirlı̀, C., Cadamuro, M., Mariotti, V., Perugorria, M. J., Bañales, J. M., & Strazzabosco, M. (2019) · Nature Reviews Gastroenterology & Hepatology · doi:10.1038/s41575-019-0156-4 · Cited by 114 · see citing works
Cystic fibrosis: An inherited disease affecting mucin-producing organs
Ehré, C., Ridley, C. E., & Thornton, D. J. (2014) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2014.03.011 · Cited by 111 · see citing works
Cystic Fibrosis–related Liver Disease
Debray, D., Narkewicz, M. R., Bodewes, F. A. J. A., Colombo, C., Housset, C., de Jonge, H. R., Jonker, J. W., Kelly, D. A., Ling, S. C., Poynard, T., et al. (2017) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0000000000001676 · Cited by 108 · see citing works
Cystic Fibrosis, CFTR, and Colorectal Cancer
Scott, P. M., Anderson, K., Singhania, M., & Cormier, R. T. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21082891 · Cited by 95 · see citing works
The Gut-Lung Axis in Cystic Fibrosis
Price, C. E., & O’Toole, G. A. (2021) · Journal of Bacteriology · doi:10.1128/jb.00311-21 · Cited by 84 · see citing works
Cftr Modulates Wnt/β-Catenin Signaling and Stem Cell Proliferation in Murine Intestine
Strubberg, A. M., Liu, J., Walker, N. M., Stefanski, C. D., MacLeod, R. J., Magness, S. T., & Clarke, L. L. (2017) · Cellular and Molecular Gastroenterology and Hepatology · doi:10.1016/j.jcmgh.2017.11.013 · Cited by 84 · see citing works
Jin, C., Xia, J., Wu, S., Tu, W., Pan, Z., Fu, Z., Wang, Y., & Jin, Y. (2017) · Toxicological Sciences · doi:10.1093/toxsci/kfx227 · Cited by 84 · see citing works
Faecal proteomics: A tool to investigate dysbiosis and inflammation in patients with cystic fibrosis
Debyser, G., Mesuere, B., Clement, L., de Weygaert, J. V., Hecke, P. V., Duytschaever, G., Aerts, M., Dawyndt, P., Boeck, K. D., Vandamme, P. A. R., et al. (2015) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2015.08.003 · Cited by 80 · see citing works
CFTR, bicarbonate, and the pathophysiology of cystic fibrosis
Borowitz, D. (2015) · Pediatric Pulmonology · doi:10.1002/ppul.23247 · Cited by 79 · see citing works
Niccum, D. E., Billings, J. L., Dunitz, J., & Khoruts, A. (2016) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2016.01.002 · Cited by 79 · see citing works
Pan, Z., Yuan, X., Tu, W., Fu, Z., & Jin, Y. (2019) · Environmental Pollution · doi:10.1016/j.envpol.2019.07.021 · Cited by 74 · see citing works
Garg, M., & Ooi, C. Y. (2017) · Current Gastroenterology Reports · doi:10.1007/s11894-017-0546-0 · Cited by 68 · see citing works
Fiorotto, R., & Strazzabosco, M. (2019) · Cellular and Molecular Gastroenterology and Hepatology · doi:10.1016/j.jcmgh.2019.04.013 · Cited by 69 · see citing works
de Freitas, M. B., Moreira, E. A. M., Tomio, C., Moreno, Y. M. F., Daltoé, F. P., Barbosa, E., Neto, N. L., Buccigrossi, V., & Guarino, A. (2018) · PLoS ONE · doi:10.1371/journal.pone.0198457 · Cited by 69 · see citing works
Bazett, M., Bergeron, M.-E., & Haston, C. K. (2016) · Scientific Reports · doi:10.1038/srep19189 · Cited by 60 · see citing works
Cystic fibrosis: Evidence for gut inflammation
Munck, À. (2014) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2014.02.005 · Cited by 59 · see citing works
Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology
Staufer, K., Halilbasic, E., Trauner, M., & Kazemi-Shirazi, L. (2014) · International Journal of Molecular Sciences · doi:10.3390/ijms150813529 · Cited by 50 · see citing works
Olivier, A. K., Gibson-Corley, K. N., & Meyerholz, D. K. (2015) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00146.2014 · Cited by 56 · see citing works
Intestinal complications of cystic fibrosis
Borowitz, D., & Gelfond, D. (2013) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0b013e3283659ef2 · Cited by 55 · see citing works
Disrupted tight junctions in the small intestine of cystic fibrosis mice
Lisle, R. C. D. (2013) · Cell and Tissue Research · doi:10.1007/s00441-013-1734-3 · Cited by 48 · see citing works
Role of vitamin D on gut microbiota in cystic fibrosis
Kanhere, M., Chassaing, B., Gewirtz, A. T., & Tangpricha, V. (2016) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/j.jsbmb.2016.11.001 · Cited by 46 · see citing works
Microbiome in the pathogenesis of cystic fibrosis and lung transplant-related disease
Cribbs, S. K., & Beck, J. M. (2016) · Translational research · doi:10.1016/j.trsl.2016.07.022 · Cited by 44 · see citing works
Bile acid homeostasis in gastrointestinal and metabolic complications of cystic fibrosis
van de Peppel, I. P., Bodewes, F. A. J. A., Verkade, H. J., & Jonker, J. W. (2018) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2018.08.009 · Cited by 43 · see citing works
Acquisition and adaptation of the airway microbiota in the early life of cystic fibrosis patients
Boutin, S., & Dalpke, A. H. (2017) · Molecular and Cellular Pediatrics · doi:10.1186/s40348-016-0067-1 · Cited by 38 · see citing works
Shin, N. R., Bose, S., Wang, J., Ansari, A., Lim, S.-K., Chin, Y., Choi, H., & Kim, H. (2017) · Frontiers in Microbiology · doi:10.3389/fmicb.2017.02271 · Cited by 39 · see citing works
Cystic fibrosis mouse model-dependent intestinal structure and gut microbiome
Bazett, M., Honeyman, L., Stefanov, A. N., Pope, C. E., Hoffman, L. R., & Haston, C. K. (2015) · Mammalian Genome · doi:10.1007/s00335-015-9560-4 · Cited by 34 · see citing works
The CF gastrointestinal microbiome: Structure and clinical impact
Rogers, G. B., Narkewicz, M. R., & Hoffman, L. R. (2016) · Pediatric Pulmonology · doi:10.1002/ppul.23544 · Cited by 32 · see citing works
Madan, J. C. (2016) · Clinical Therapeutics · doi:10.1016/j.clinthera.2016.02.008 · Cited by 31 · see citing works
Burke, D. G., Harrison, M. J., Fleming, C., McCarthy, M. M., Shortt, C., Sulaiman, I., Murphy, D. M., Eustace, J. A., Shanahan, F., Hill, C., et al. (2016) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2016.09.008 · Cited by 31 · see citing works
Patients with cystic fibrosis have a high carriage rate of non-toxigenic Clostridium difficile
Bauer, M. P., Farid, A., Bakker, M., Hoek, R. A. S., Kuijper, E. J., & van Dissel, J. T. (2013) · Clinical Microbiology and Infection · doi:10.1111/1469-0691.12439 · Cited by 28 · see citing works
Pathophysiological role of ion channels and transporters in gastrointestinal mucosal diseases
Deng, Z., Zhao, Y., Ma, Z., Zhang, M., Wang, H., Yi, Z., Tuo, B., Li, T., & Liu, X. (2021) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-021-04011-5 · Cited by 29 · see citing works
Hydrogen peroxide scavenger, catalase, alleviates ion transport dysfunction in murine colitis
Barrett, K. E., & McCole, D. F. (2016) · Clinical and Experimental Pharmacology and Physiology · doi:10.1111/1440-1681.12646 · Cited by 25 · see citing works
Transcriptome analysis of the distal small intestine of Cftr null mice
Ikpa, P. T., Meijsen, K. F., Nieuwenhuijze, N. D. A., Dulla, K., de Jonge, H. R., & Bijvelds, M. J. C. (2019) · Genomics · doi:10.1016/j.ygeno.2019.06.028 · Cited by 23 · see citing works
Hardouin, P., Chiron, R., Marchandin, H., Armengaud, J., & Grenga, L. (2021) · Genes · doi:10.3390/genes12060892 · Cited by 22 · see citing works
Kelly, J., Al-Rammahi, M. A., Daly, K., Flanagan, P. K., Urs, A. N., Cohen, M. C., Stefano, G. D., Bijvelds, M. J. C., Sheppard, D. N., de Jonge, H. R., et al. (2022) · Scientific Reports · doi:10.1038/s41598-022-10328-3 · Cited by 23 · see citing works
Yeh, K. M., Johansson, O., Le, H., Rao, K., Markus, I., Perera, D. S., Lubowski, D. Z., King, D. W., Zhang, L., Chen, H., et al. (2019) · Journal of Gastroenterology · doi:10.1007/s00535-019-01610-9 · Cited by 21 · see citing works
Dunwoody, R., Steel, A., Landy, J., & Simmonds, N. J. (2017) · Paediatric Respiratory Reviews · doi:10.1016/j.prrv.2017.03.003 · Cited by 19 · see citing works
Impaired Intestinal Farnesoid X Receptor Signaling in Cystic Fibrosis Mice
Ikpa, P. T., Doktorova, M., Meijsen, K. F., Nieuwenhuijze, N. D. A., Verkade, H. J., Jonker, J. W., de Jonge, H. R., & Bijvelds, M. J. C. (2019) · Cellular and Molecular Gastroenterology and Hepatology · doi:10.1016/j.jcmgh.2019.08.006 · Cited by 19 · see citing works
Lisle, R. C. D. (2015) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0000000000001030 · Cited by 15 · see citing works
Probiotics Administration in Cystic Fibrosis: What Is the Evidence?
Esposito, S., Testa, I., Zani, E. M., Cunico, D., Torelli, L., Grandinetti, R., Fainardi, V., Pisi, G., & Principi, N. (2022) · Nutrients · doi:10.3390/nu14153160 · Cited by 15 · see citing works
Animal models for cystic fibrosis liver disease (CFLD)
Fiorotto, R., Amenduni, M., Mariotti, V., Cadamuro, M., Fabris, L., Spirlı̀, C., & Strazzabosco, M. (2018) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2018.07.026 · Cited by 15 · see citing works
Prebiotic Properties of Galursan HF 7K on Mouse Gut Microbiota
Engevik, M. A., Faletti, C. J., Paulmichl, M., & Worrell, R. T. (2013) · Cellular Physiology and Biochemistry · doi:10.1159/000356631 · Cited by 13 · see citing works
Cortes‐Santiago, N., Leung, D. H., Castro, E., Finegold, M. J., Wu, H., & Patel, K. R. (2018) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0000000000002154 · Cited by 12 · see citing works
Guo, J., & Liyang, Y. A. N. G. (2024) · Bioscience of Microbiota Food and Health · doi:10.12938/bmfh.2023-100 · Cited by 10 · see citing works
Song, A., Shen, N., Gan, C., Luo, C., Luo, C., Wang, J., Cao, Q. N., & Chen, J. (2021) · Translational Pediatrics · doi:10.21037/tp-20-208 · Cited by 10 · see citing works
Intestinal Dysbiosis in Young Cystic Fibrosis Rabbits
Liang, X., Bouhamdan, M., Hou, X., Zhang, K., Song, J. W., Hao, K., Jin, J., Zhang, Z., & Xu, J. (2021) · Journal of Personalized Medicine · doi:10.3390/jpm11020132 · Cited by 10 · see citing works
The human microbiome: opportunities and challenges for clinical care
Rogers, G. B. (2014) · Internal Medicine Journal · doi:10.1111/imj.12650 · Cited by 10 · see citing works
AMPK mediates inhibition of electrolyte transport and NKCC1 activity by reactive oxygen species
King, S. J., Bunz, M., Chappell, A. E., Scharl, M. M., Docherty, M. J., Jung, B., Lytle, C. Y., & McCole, D. F. (2019) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00317.2018 · Cited by 10 · see citing works
Goblet cell hyperplasia is not epithelial-autonomous in the Cftr knockout intestine
Walker, N. M., Liu, J., Young, S. M., Woode, R. A., & Clarke, L. L. (2021) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00290.2021 · Cited by 10 · see citing works
ABCC7/CFTR Expression Is Associated with the Clinical Course of Ulcerative Colitis Patients
Villeda-Ramírez, M. A., Meza-Guillén, D., Barreto‐Zúñiga, R., & Yamamoto-Furusho, J. K. (2021) · Gastroenterology Research and Practice · doi:10.1155/2021/5536563 · Cited by 9 · see citing works
Scull, C. E., Luo, M., Jennings, S., Taylor, C. M., & Wang, G. (2022) · Communications Biology · doi:10.1038/s42003-022-04101-5 · Cited by 9 · see citing works
Swimming Through the Gut: Implications of Fluid Transport on the Microbiome
Colgan, S. P. (2013) · Digestive Diseases and Sciences · doi:10.1007/s10620-013-2575-3 · Cited by 8 · see citing works
Sivapiromrat, A. K., Suppakitjanusant, P., Wang, Y., Hu, C., Binongo, J., Hunt, W. R., Weinstein, S., Jathal, I., Alvarez, J. A., Chassaing, B., et al. (2024) · Contemporary Clinical Trials Communications · doi:10.1016/j.conctc.2024.101278 · Cited by 6 · see citing works
Surveillance of Colorectal Cancer (CRC) in Cystic Fibrosis (CF) Patients
Ingravalle, F., Casella, G., Ingravalle, A., Monti, C. M., Salvatore, F. D., Stillitano, D. M., & Villanacci, V. (2021) · Gastrointestinal Disorders · doi:10.3390/gidisord3020009 · Cited by 6 · see citing works
Insights on the Pathogenesis of Mycobacterium abscessus Infection in Patients with Cystic Fibrosis
Basher, M., Gur, M., & Meir, M. (2025) · Journal of Clinical Medicine · doi:10.3390/jcm14103492 · Cited by 5 · see citing works
Young, S. M., Woode, R. A., Williams, E., Ericsson, A. C., & Clarke, L. L. (2023) · Physiological Genomics · doi:10.1152/physiolgenomics.00077.2023 · Cited by 5 · see citing works
Woode, R. A., Strubberg, A. M., Liu, J., Walker, N. M., & Clarke, L. L. (2024) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.00211.2022 · Cited by 4 · see citing works
Liu, Z., Zeinalzadeh, Z., Huang, T., Han, Y., Peng, L., Wang, D., Zhou, Z., Ousmane, D., & Wang, J. (2024) · Heliyon · doi:10.1016/j.heliyon.2024.e37053 · Cited by 4 · see citing works
Caverly, L. J., Hoffman, L. R., & Zemanick, E. T. (2022) · Respiratory medicine · doi:10.1007/978-3-030-87104-8_6 · Cited by 4 · see citing works
(2013) · Pediatric Pulmonology · doi:10.1002/ppul.22896 · Cited by 2 · see citing works
Zhou, H., Wu, H., Chen, Y., Zou, W., Lü, W., & He, Y. (2021) · Frontiers in Microbiology · doi:10.3389/fmicb.2021.712212 · Cited by 2 · see citing works
Ageing with cystic fibrosis: Challenges ahead
Spelier, S., van der Wijst, M. I. M., Fajac, I., & Downey, D. G. (2026) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2025.12.015 · Cited by 1 · see citing works
Lung and Gut Microbiota Interactions with Air Pollution and Aging in Human Chronic Diseases
Mohammed, A. N., & Yadav, J. S. (2023) · Healthy ageing and longevity · doi:10.1007/978-3-031-14023-5_11 · Cited by 1 · see citing works
Pharmacological and Clinical Treatment of Irritable Bowel Syndrome
Sałaga, M., & Mosińska, P. (2016) · Springer eBooks · doi:10.1007/978-3-319-49016-8_4
Cystic fibrosis defects in intestinal bile acid and guanylin signaling
Ikpa, P. T. (2017) · RePub (Erasmus University Rotterdam)
de Jonge, H. R. (2018) · Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature · doi:10.3410/f.733082086.793545697
Abely, M. (2013) · Revue des Maladies Respiratoires Actualités · doi:10.1016/s1877-1203(13)70490-7
Human microbiome and respiratory diseases
Pathak, A., Roy, M., Puno, G., Pandiyarajan, A. P., Baraskar, S. M., & Chaudhary, K. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-15435-5.00009-8
Cystic Fibrosis Impact on Cellular Function
Rao, P. (2014) · Carroll Collected (John Carroll University)
- Authors
- Lynch, S. V., Goldfarb, K. C., Wild, Y. K., Kong, W., De Lisle, R. C., & Brodie, E. L.
- Affiliations
- University of California, San Francisco (US)
- Lawrence Berkeley National Laboratory (US)
- Corresponding author
- Lynch, Susan V.
- Venue
- Gut Microbes
- Publisher
- Informa UK Limited
- Published
- 2013-01
- Volume
- 4
- Issue
- 1
- Pages
- 41-47
- ISSN
- 1949-0976 (ISSN-L)
- 1949-0984
- PMC
- PMC3555885
- OpenAlex
- W2020459103
- Cited by
- 87
- References
- 39
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 5 · 2023: 3 · 2022: 4 · 2021: 9 · 2020: 4 · 2019: 8 · 2018: 6 · 2017: 11 · 2016: 11 · 2015: 8 · 2014: 8 · 2013: 8
- FWCI
- 5.23
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Gut Microbes (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- Kenneth Rainin Foundation
- National Institute of Allergy and Infectious Diseases (R21AI083479, AI083479)
- National Institute of Diabetes and Digestive and Kidney Diseases (T32DK007762)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Gut microbiota and health
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal motility and disorders
- Keywords
- cystic fibrosis, CFTR knockout mice, gut microbiota, bacterial diversity, antibiotic therapy, mycobacteria, Bacteroides fragilis, Enterobacteriaceae, Clostridiaceae, Lactobacillaceae, Bifidobacteriaceae, Acinetobacter lwoffii, intestinal motility, intestinal inflammation, immunomodulation
- MeSH terms
- Animals, Bacteria, Cystic Fibrosis, Disease Models, Animal, Female, Male, Mice, Knockout, Cystic Fibrosis Transmembrane Conductance Regulator, Gastrointestinal Tract, Biodiversity, Microarray Analysis, Mice, Biota, Bacterial Load
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