Literature DB >> 25591863

Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology.

Alicia K Olivier1, Katherine N Gibson-Corley1, David K Meyerholz2.   

Abstract

Multiple organ systems, including the gastrointestinal tract, pancreas, and hepatobiliary systems, are affected by cystic fibrosis (CF). Many of these changes begin early in life and are difficult to study in young CF patients. Recent development of novel CF animal models has expanded opportunities in the field to better understand CF pathogenesis and evaluate traditional and innovative therapeutics. In this review, we discuss manifestations of CF disease in gastrointestinal, pancreatic, and hepatobiliary systems of humans and animal models. We also compare the similarities and limitations of animal models and discuss future directions for modeling CF.
Copyright © 2015 the American Physiological Society.

Entities:  

Keywords:  cystic fibrosis; cystic fibrosis transmembrane conductance regulator; gastrointestinal tract; liver; pancreas

Mesh:

Substances:

Year:  2015        PMID: 25591863      PMCID: PMC4360044          DOI: 10.1152/ajpgi.00146.2014

Source DB:  PubMed          Journal:  Am J Physiol Gastrointest Liver Physiol        ISSN: 0193-1857            Impact factor:   4.052


  133 in total

1.  Neonatal spontaneous colonic perforation due to cystic fibrosis.

Authors:  M M F Siddiqui; D M Burge
Journal:  Pediatr Surg Int       Date:  2008-04-26       Impact factor: 1.827

2.  Generation of a conditional null allele for Cftr in mice.

Authors:  Craig A Hodges; Calvin U Cotton; Mark R Palmert; Mitchell L Drumm
Journal:  Genesis       Date:  2008-10       Impact factor: 2.487

Review 3.  Pancreatic dysfunction in cystic fibrosis occurs as a result of impairments in luminal pH, apical trafficking of zymogen granule membranes, and solubilization of secretory enzymes.

Authors:  G A Scheele; S I Fukuoka; H F Kern; S D Freedman
Journal:  Pancreas       Date:  1996-01       Impact factor: 3.327

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Journal:  N Engl J Med       Date:  1995-02-23       Impact factor: 91.245

5.  Fibrogenesis in pediatric cholestatic liver disease: role of taurocholate and hepatocyte-derived monocyte chemotaxis protein-1 in hepatic stellate cell recruitment.

Authors:  Grant A Ramm; Ross W Shepherd; Anita C Hoskins; Sonia A Greco; Agnieszka D Ney; Tamara N Pereira; Kim R Bridle; James D Doecke; Peter J Meikle; Bruno Turlin; Peter J Lewindon
Journal:  Hepatology       Date:  2009-02       Impact factor: 17.425

6.  Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.

Authors:  J H Poulsen; H Fischer; B Illek; T E Machen
Journal:  Proc Natl Acad Sci U S A       Date:  1994-06-07       Impact factor: 11.205

7.  Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice.

Authors:  R C De Lisle
Journal:  Am J Physiol       Date:  1995-04

8.  Ion transport across the jejunum in normal and cystic fibrosis mice.

Authors:  B R Grubb
Journal:  Am J Physiol       Date:  1995-03

9.  Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR.

Authors:  L Zhou; C R Dey; S E Wert; M D DuVall; R A Frizzell; J A Whitsett
Journal:  Science       Date:  1994-12-09       Impact factor: 47.728

10.  Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.

Authors:  Christopher S Rogers; David A Stoltz; David K Meyerholz; Lynda S Ostedgaard; Tatiana Rokhlina; Peter J Taft; Mark P Rogan; Alejandro A Pezzulo; Philip H Karp; Omar A Itani; Amanda C Kabel; Christine L Wohlford-Lenane; Greg J Davis; Robert A Hanfland; Tony L Smith; Melissa Samuel; David Wax; Clifton N Murphy; August Rieke; Kristin Whitworth; Aliye Uc; Timothy D Starner; Kim A Brogden; Joel Shilyansky; Paul B McCray; Joseph Zabner; Randall S Prather; Michael J Welsh
Journal:  Science       Date:  2008-09-26       Impact factor: 47.728

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  22 in total

Review 1.  Animal models of biliary injury and altered bile acid metabolism.

Authors:  Valeria Mariotti; Mario Strazzabosco; Luca Fabris; Diego F Calvisi
Journal:  Biochim Biophys Acta Mol Basis Dis       Date:  2017-07-11       Impact factor: 5.187

Review 2.  Stem cell-derived organoids to model gastrointestinal facets of cystic fibrosis.

Authors:  Meike Hohwieler; Lukas Perkhofer; Stefan Liebau; Thomas Seufferlein; Martin Müller; Anett Illing; Alexander Kleger
Journal:  United European Gastroenterol J       Date:  2016-09-21       Impact factor: 4.623

Review 3.  CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis.

Authors:  Péter Hegyi; Michael Wilschanski; Shmuel Muallem; Gergely L Lukacs; Miklós Sahin-Tóth; Aliye Uc; Michael A Gray; Zoltán Rakonczay; József Maléth
Journal:  Rev Physiol Biochem Pharmacol       Date:  2016       Impact factor: 5.545

Review 4.  Animal and model systems for studying cystic fibrosis.

Authors:  Bradley H Rosen; Marc Chanson; Lara R Gawenis; Jinghua Liu; Aderonke Sofoluwe; Alice Zoso; John F Engelhardt
Journal:  J Cyst Fibros       Date:  2017-09-19       Impact factor: 5.482

Review 5.  The CF gastrointestinal microbiome: Structure and clinical impact.

Authors:  Geraint B Rogers; Michael R Narkewicz; Lucas R Hoffman
Journal:  Pediatr Pulmonol       Date:  2016-10

Review 6.  Animal models for cystic fibrosis liver disease (CFLD).

Authors:  Romina Fiorotto; Mariangela Amenduni; Valeria Mariotti; Massimiliano Cadamuro; Luca Fabris; Carlo Spirli; Mario Strazzabosco
Journal:  Biochim Biophys Acta Mol Basis Dis       Date:  2018-07-30       Impact factor: 5.187

7.  Liver reserve function assessment by acoustic radiation force impulse imaging.

Authors:  Xiao-Lan Sun; Li-Wei Liang; Hui Cao; Qiong Men; Ke-Zhu Hou; Zhen Chen; Ya-E Zhao
Journal:  World J Gastroenterol       Date:  2015-08-28       Impact factor: 5.742

8.  Role of CTGF gene promoter methylation in the development of hepatic fibrosis.

Authors:  Cuicui Shi; Guangming Li; Yanyan Tong; Yilin Deng; Jiangao Fan
Journal:  Am J Transl Res       Date:  2016-01-15       Impact factor: 4.060

Review 9.  Pancreatic pathophysiology in cystic fibrosis.

Authors:  Katherine N Gibson-Corley; David K Meyerholz; John F Engelhardt
Journal:  J Pathol       Date:  2015-10-01       Impact factor: 7.996

Review 10.  Lessons learned from the cystic fibrosis pig.

Authors:  David K Meyerholz
Journal:  Theriogenology       Date:  2016-04-21       Impact factor: 2.740

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