Literature DB >> 15496606

Fic1 is expressed at apical membranes of different epithelial cells in the digestive tract and is induced in the small intestine during postnatal development of mice.

Saskia W C van Mil1, Masja M van Oort, Inge E T van den Berg, Ruud Berger, Roderick H J Houwen, Leo W J Klomp.   

Abstract

Mutations in ATP8B1 are associated with FIC1 disease, an autosomal recessive disorder in which intrahepatic cholestasis is the predominant manifestation. ATP8B1 encodes FIC1, which is expressed in several tissues, most prominently in the intestine, pancreas, and stomach and, to a much lesser extent, in the liver. In this study, Fic1 localization and expression during postnatal development was examined in healthy mice. Immunoblot and RT-PCR analysis indicated Fic1 is expressed abundantly in regions of the adult gastrointestinal tract of humans and mice. Immunohistochemistry revealed that Fic1 was localized to the apical membranes of enterocytes, pancreatic acinar cells, gastric pit epithelial cells, and hepatocytes and cholangiocytes. Subsequent analysis of early postnatal expression revealed that Fic1 expression in the small intestine was limited or absent at the age of 7 and 14 d and increased significantly with maturation. In contrast, pancreatic, hepatic, and gastric Fic1 expression was not diminished during the first 3 wk of postnatal development. In conclusion, these data show that Fic1 is expressed in a tissue-specific and developmentally regulated fashion at the apical membranes of epithelial cells. We speculate that the developing bile salt pool in the maturing intestine accounts for the increase in Fic1 protein expression in this tissue.

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Year:  2004        PMID: 15496606     DOI: 10.1203/01.PDR.0000145564.06791.D1

Source DB:  PubMed          Journal:  Pediatr Res        ISSN: 0031-3998            Impact factor:   3.756


  16 in total

1.  Differences in presentation and progression between severe FIC1 and BSEP deficiencies.

Authors:  Ludmila Pawlikowska; Sandra Strautnieks; Irena Jankowska; Piotr Czubkowski; Karan Emerick; Anthony Antoniou; Catherine Wanty; Bjorn Fischler; Emmanuel Jacquemin; Sami Wali; Samra Blanchard; Inge-Merete Nielsen; Billy Bourke; Shirley McQuaid; Florence Lacaille; Jane A Byrne; Albertien M van Eerde; Kaija-Leena Kolho; Leo Klomp; Roderick Houwen; Peter Bacchetti; Steven Lobritto; Vera Hupertz; Patricia McClean; Giorgina Mieli-Vergani; Benjamin Shneider; Antal Nemeth; Etienne Sokal; Nelson B Freimer; A S Knisely; Philip Rosenthal; Peter F Whitington; Joanna Pawlowska; Richard J Thompson; Laura N Bull
Journal:  J Hepatol       Date:  2010-04-13       Impact factor: 25.083

2.  The membrane protein ATPase class I type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptor.

Authors:  Tamara Frankenberg; Tamir Miloh; Frank Y Chen; Meena Ananthanarayanan; An-Qiang Sun; Natarajan Balasubramaniyan; Irwin Arias; Kenneth D R Setchell; Frederick J Suchy; Benjamin L Shneider
Journal:  Hepatology       Date:  2008-12       Impact factor: 17.425

Review 3.  Genetics of familial intrahepatic cholestasis syndromes.

Authors:  S W C van Mil; R H J Houwen; L W J Klomp
Journal:  J Med Genet       Date:  2005-06       Impact factor: 6.318

4.  CDC50 proteins are critical components of the human class-1 P4-ATPase transport machinery.

Authors:  Susanne Bryde; Hanka Hennrich; Patricia M Verhulst; Philippe F Devaux; Guillaume Lenoir; Joost C M Holthuis
Journal:  J Biol Chem       Date:  2010-10-20       Impact factor: 5.157

5.  FIC1-mediated stimulation of FXR activity is decreased with PFIC1 mutations in HepG2 cells.

Authors:  Saori Koh; Tappei Takada; Ikuya Kukuu; Hiroshi Suzuki
Journal:  J Gastroenterol       Date:  2009-04-21       Impact factor: 7.527

6.  Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content.

Authors:  Coen C Paulusma; D Rudi de Waart; Cindy Kunne; Kam S Mok; Ronald P J Oude Elferink
Journal:  J Biol Chem       Date:  2009-02-19       Impact factor: 5.157

7.  ATP8B1 is essential for maintaining normal hearing.

Authors:  Janneke M Stapelbroek; Theo A Peters; Denis H A van Beurden; Jo H A J Curfs; Anneke Joosten; Andy J Beynon; Bibian M van Leeuwen; Lieke M van der Velden; Laura Bull; Ronald P Oude Elferink; Bert A van Zanten; Leo W J Klomp; Roderick H J Houwen
Journal:  Proc Natl Acad Sci U S A       Date:  2009-05-28       Impact factor: 11.205

Review 8.  P4 ATPases: flippases in health and disease.

Authors:  Vincent A van der Mark; Ronald P J Oude Elferink; Coen C Paulusma
Journal:  Int J Mol Sci       Date:  2013-04-11       Impact factor: 5.923

9.  ATP8B1 gene expression is driven by a housekeeping-like promoter independent of bile acids and farnesoid X receptor.

Authors:  Dita Cebecauerová; Sandra S Strautnieks; Jane A Byrne; Milan Jirsa; Richard J Thompson
Journal:  PLoS One       Date:  2012-12-10       Impact factor: 3.240

10.  An unexpectedly high degree of specialization and a widespread involvement in sterol metabolism among the C. elegans putative aminophospholipid translocases.

Authors:  Nicholas N Lyssenko; Yana Miteva; Simon Gilroy; Wendy Hanna-Rose; Robert A Schlegel
Journal:  BMC Dev Biol       Date:  2008-10-02       Impact factor: 1.978

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