Altered intestinal bile salt biotransformation in a cystic fibrosis (Cftr−/−) mouse model with hepato-biliary pathology Frank A.J.A. Bodewes, Mariëtte.

Slides:

Advertisements

Similar presentations

Use of the insulin pump in treat cystic fibrosis related diabetes Dana S. Hardin, Julie Rice, Mark Rice, Randall Rosenblatt Journal of Cystic Fibrosis.

Advertisements

Volume 114, Issue 5, Pages (May 1998)

C. P. B. van der Ploeg, M. E. van den Akker-van Marle, A. M. M

Treatment compliance in children and adults with Cystic Fibrosis

Analysis of the CFTR gene in Iranian cystic fibrosis patients: Identification of eight novel mutations Reza Alibakhshi, Roya Kianishirazi, Jean-Jacques.

Left ventricular and aortic dysfunction in cystic fibrosis mice

P. Whitaker, N. Shaw, J. Gooi, C. Etherington, S. Conway, D. Peckham

The immediate effect of musculoskeletal physiotherapy techniques and massage on pain and ease of breathing in adults with cystic fibrosis Annemarie Lee,

Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function Fredrick Van Goor, Haihui Yu, Bill.

Dietary supplementation with multiple micronutrients: No beneficial effects in pediatric cystic fibrosis patients Johanna H. Oudshoorn, Peter H.C. Klijn,

Volume 42, Issue 4, Pages (April 2005)

HbA1c as a screening tool for cystic fibrosis related diabetes

Risk factors for age at initial Pseudomonas acquisition in the cystic fibrosis epic observational cohort Margaret Rosenfeld, Julia Emerson, Sharon McNamara,

Volume 136, Issue 2, Pages e1 (February 2009)

How does heart rate recovery after sub-maximal exercise correlate with maximal exercise testing in children with CF? Sarah P. Cohen, David M. Orenstein

Impact of storage conditions on metabolite profiles of sputum samples from persons with cystic fibrosis Jiangchao Zhao, Charles R. Evans, Lisa A. Carmody,

Megan R. Nelson, Craig R. Adamski, Audrey Tluczek

Amoxicillin–clavulanic acid resistance in fecal Enterobacteriaceae from patients with cystic fibrosis and healthy siblings Gwen Duytschaever, Geert Huys,

Samantha A. Woodruff, Marci K. Sontag, Frank J. Accurso, Ronald J

Urinary incontinence in 9��16��year olds with cystic fibrosis compared to other respiratory conditions and a normal group W.J. Browne, C.J. Wood, M.

Measures of body habitus are associated with lung function in adults with cystic fibrosis: A population-based study Doug L. Forrester, Alan J. Knox,

Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy

Cirrhosis and other liver disease in cystic fibrosis

Expression of the inflammatory regulator A20 correlates with lung function in patients with cystic fibrosis Catriona Kelly, Mark T. Williams, J. Stuart.

Controlled clinical trials in cystic fibrosis — are we doing better?

D. Stevens, P.J. Oades, N. Armstrong, C.A. Williams

Claire J. Tipping, Rebecca L. Scholes, Narelle S. Cox

The Journal of Cystic Fibrosis: Sunny perspectives

Vitamin A and lung function in CF

Use of the insulin pump in treat cystic fibrosis related diabetes

A.H. Gifford Journal of Cystic Fibrosis

M. Wouthuyzen-Bakker, F.A.J.A. Bodewes, H.J. Verkade

Fatty acids in blood and intestine following docosahexaenoic acid supplementation in adults with cystic fibrosis Jacqueline A. Jumpsen, Neil E. Brown,

Demographics of glucose metabolism in cystic fibrosis

Cystic fibrosis related diabetes in an extremely young patient

Agreement of bioelectric impedance analysis and dual-energy X-ray absorptiometry for body composition evaluation in adults with cystic fibrosis S. Ziai,

Inhalation solutions — Which ones may be mixed

Physiologic endpoints for clinical studies for cystic fibrosis

Antonia V. Bennett, Donald L. Patrick, James F. Lymp, Todd C

Characterisation of mutations and genotype–phenotype correlation in cystic fibrosis: Experience from India Shivaram S. Shastri, Madhulika Kabra, Sushil.

Association between practice patterns and body mass index percentile in infants and young children with cystic fibrosis Raj Padman, Lloyd N. Werk, Gabriela.

Sophia L. Markantonis, Anna Katelari, Eleni Pappa, Stavros Doudounakis

Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor Meredith C. Fidler, Jack Beusmans, Paul Panorchan,

Preliminary report of the 13C-mixed triglyceride breath test to assess timing of pancreatic enzyme replacement therapy in children with cystic fibrosis

Anastasios Lekkas, Khin M. Gyi, Margaret E. Hodson

Prevalence and risk factors for recovery of filamentous fungi in individuals with cystic fibrosis Christopher R. Sudfeld, Elliott C. Dasenbrook, William.

Chee Y. Ooi, Peter R. Durie Journal of Cystic Fibrosis

Novel concepts in evaluating antimicrobial therapy for bacterial lung infections in patients with cystic fibrosis Geraint B. Rogers, Lucas R. Hoffman,

Effect of ivacaftor in patients with advanced cystic fibrosis and a G551D-CFTR mutation: Safety and efficacy in an expanded access program in the United.

Segregation of children with CF diagnosed via newborn screening and acquisition of Pseudomonas aeruginosa Karen O. McKay, Peter J. Cooper, Peter P. van.

Higher risk of hospitalization among females with cystic fibrosis

Trends in pathogens colonising the respiratory tract of adult patients with cystic fibrosis, 1985–2005 F.A. Millar, N.J. Simmonds, M.E. Hodson Journal.

Results of antibiotic susceptibility testing do not influence clinical outcome in children with cystic fibrosis M.N. Hurley, A.H. Amin Ariff, C. Bertenshaw,

Lung clearance index during hospital admission in school-age children with cystic fibrosis Liam Welsh, Christopher Nesci, Haily Tran, Marisol Tomai,

Determining presence of lung disease in young children with cystic fibrosis: Lung clearance index, oxygen saturation and cough frequency E.M. Bakker,

Modification of the salivary secretion assay in F508del mice — The murine equivalent of the human sweat test Karoline Droebner, Peter Sandner Journal.

J.E. Spahr, R.B. Love, M. Francois, K. Radford, K.C. Meyer

James H. Hull, Rachel Garrod, Timothy B. Ho, Ronald K. Knight, John R

Daniel J. Smith, Gregory J. Anderson, Scott C. Bell, David W. Reid

Risk factors for Mycobacterium abscessus infection in cystic fibrosis patients; a case– control study Maarten Verregghen, Harry G. Heijerman, Monique.

L. Uppaluri, S.J. England, T.F. Scanlin Journal of Cystic Fibrosis

Gut dysbiosis in cystic fibrosis

CFTR modulators and pregnancy: Our work has only just begun

‘Peeling paint’ dermatitis as a presenting sign of cystic fibrosis

Lutz Goldbeck, Sven Zerrer, Tim G. Schmitz Journal of Cystic Fibrosis

Khin M. Gyi, Margaret E. Hodson, Magdi Y. Yacoub

David Adeboyeku, Sandra Scott, Margaret E. Hodson

Elisabeth P. Dellon, Margaret W. Leigh, James R. Yankaskas, Terry L

Absence of a gender gap in survival

R. D. Gray, M. Imrie, A. C. Boyd, D. Porteous, J. A. Innes, A. P

Presentation transcript:

Altered intestinal bile salt biotransformation in a cystic fibrosis (Cftr−/−) mouse model with hepato-biliary pathology Frank A.J.A. Bodewes, Mariëtte Y.M. van der Wulp, Satti Beharry, Marcela Doktorova, Rick Havinga, Renze Boverhof, M. James Phillips, Peter R. Durie, Henkjan J. Verkade Journal of Cystic Fibrosis Volume 14, Issue 4, Pages 440-446 (July 2015) DOI: 10.1016/j.jcf.2014.12.010 Copyright © 2014 Terms and Conditions

Fig. 1 Bile production and bile salt secretion parameters of Cftr+/+ and Cftr−/− mice. Biliary bile flow (A), bile salt concentration (B) bile salt secretion rate (C) and bile salt secretion rate (BSSR) vs. bile flow in Cftr knockout mice (Cftr−/−) and control littermates (Cftr+/+). Panel D shows the correlation between all individual BSSR vs. bile flow data within both groups. Here there is a significant correlation between BSSR and bile flow in Cftr+/+ (Spearman’s Rho 0.394, P=0.042), as well as in Cftr−/− mice (Spearman’s Rho=0.762, P=0.000). Data are presented as means±SEM of n=9 mice per group. *P-value<0.05. Journal of Cystic Fibrosis 2015 14, 440-446DOI: (10.1016/j.jcf.2014.12.010) Copyright © 2014 Terms and Conditions

Fig. 2 Biliary bile salt profile of Cftr+/+ and Cftr−/− mice. Biliary bile salt profiles (A). Abbreviations: CA=cholic acid, DCA=deoxycholic acid, CDCA=chenodeoxycholic acid, HDCA=hyodeoxycholic acid, UDCA=ursodeoxycholic acid, UCA=ursocholic acid, MCA=muricholic acid. Data are presented as a percentage of different bile salt species (n=6–7 mice per group). Relative distribution between primary bile salt content and secondary bile salt (B). Primary bile salt content is decreased in Cftr−/− mice, whereas secondary bile salt content is increased (P=0.004). Heuman index (C) of the total biliary bile salts representing the hydrophobicity of bile salts. Journal of Cystic Fibrosis 2015 14, 440-446DOI: (10.1016/j.jcf.2014.12.010) Copyright © 2014 Terms and Conditions

Fig. 3 Biliary phospholipid of Cftr+/+ and Cftr−/− mice. Phospholipid concentration (A) the phospholipid secretion vs. bile salt secretion rate (B) and phospholipid to bile salt ratio (C) in Cftr knockout mice (Cftr−/−) and control littermates (Cftr+/+), n=9 mice per group. If appropriate data presented as means±SEM of n=9 mice per group. Journal of Cystic Fibrosis 2015 14, 440-446DOI: (10.1016/j.jcf.2014.12.010) Copyright © 2014 Terms and Conditions

Fig. 4 Fecal bile salt excretion and fecal bile salt profile of Cftr+/+ and Cftr−/− mice. Total bile salt excretion (A), presented as means±SEM. Bile salt profiles (B), data are presented as a percentage of different bile salt species present in bile. Abbreviations: CA=cholic acid, DCA=deoxycholic acid, CDCA=chenodeoxycholic acid, HDCA=hyodeoxycholic acid, UDCA=ursodeoxycholic acid, UCA=ursocholic acid, MCA=muricholic acid. n=9 mice per group. *P-value<0.05. Journal of Cystic Fibrosis 2015 14, 440-446DOI: (10.1016/j.jcf.2014.12.010) Copyright © 2014 Terms and Conditions

Fig. 5 Fecal bacterial content of Cftr+/+ and Cftr−/− mice. Fecal content (bacteria per gram feces) of Bacteroides (A) Lactobacillus (B) and Clostridium coccoides (C) based on microbial DNA measurement. Data are presented as means±SEM of n=6–9 mice per group. *P-value<0.05. Journal of Cystic Fibrosis 2015 14, 440-446DOI: (10.1016/j.jcf.2014.12.010) Copyright © 2014 Terms and Conditions