title: The inv mouse as an experimental model of biliary atresia.
abstract: OBJECTIVE
  The causation of biliary atresia (BA) remains unclear. However, ductal plate malformation (DPM), maldevelopment of the intrahepatic bile ducts, is 1 of the preferred theories. The inv homozygous mouse (inv mouse), created by insertional mutagenesis, shows situs inversus and jaundice. This study investigated whether the inv mouse could be an experimental model of human BA.
  
  
  METHODS
  In the inv mice (n = 12) and wild-type littermates (n = 12), we examined the liver function and morphologic changes in the biliary tract through serum biochemical study and morphological study.
  
  
  RESULTS
  The level of serum total and conjugated bilirubin in the inv mouse was 8.1 +/- 3.8 and 4.4 +/- 2.4 mg/dL, respectively, significantly higher than in the wild type. Macroscopically, 11 (92%) of 12 inv mice had situs inversus, and 3 (25%) of 12 mice had preduodenal portal vein. Histologically, the continuity of the extrahepatic bile duct was preserved. However, DPM, showing proliferative biliary epithelium around the intrahepatic portal vein, was found in the liver of the inv mouse.
  
  
  CONCLUSIONS
  In the inv mouse, the pathologic changes in DPM were found in the intrahepatic biliary system, which were observed in some clinical cases of BA. Therefore, the intrahepatic biliary system of the inv mouse could be an experimental model of human BA with DPM.
authors.affiliation: Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan. sshima@koto.kpu-m.ac.jp
authors.forename: Shinichi
authors.initials: S
authors.lastname: Shimadera
authors.forename: Naomi
authors.initials: N
authors.lastname: Iwai
authors.forename: Eiichi
authors.initials: E
authors.lastname: Deguchi
authors.forename: Osamu
authors.initials: O
authors.lastname: Kimura
authors.forename: Shigehisa
authors.initials: S
authors.lastname: Fumino
authors.forename: Takahiko
authors.initials: T
authors.lastname: Yokoyama
mesh_terms: D000818:Animals; D001652:Bile Ducts / Q000378:metabolism / Q000473:pathology; D001656:Biliary Atresia / Q000378:metabolism / Q000473:pathology; D001663:Bilirubin / Q000097:blood; D004195:Disease Models, Animal; D005260:Female; D041981:Gastrointestinal Tract / Q000002:abnormalities; D007633:Keratins; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D016254:Mutagenesis, Insertional; D012857:Situs Inversus / Q000150:complications
chemical_list: D007633:Keratins; D001663:Bilirubin