Hydrophilic bile acids prevent liver damage caused by lack of biliary phospholipid in Mdr2^-/- mice.

ABSTRACT: Bile acid imbalance causes progressive familial intrahepatic cholestasis type 2 (PFIC2) or type 3 (PFIC3), severe liver diseases associated with genetic defects in the biliary bile acid transporter bile salt export pump (BSEP; ABCB11) or phosphatidylcholine transporter multidrug resistance protein 3 (MDR3; ABCB4), respectively. Mdr2^-/- mice (a PFIC3 model) develop progressive cholangitis, ductular proliferation, periportal fibrosis, and hepatocellular carcinoma (HCC) because the nonmicelle-bound bile acids in the bile of these mice are toxic. We asked whether the highly hydrophilic bile acids generated by Bsep^-/- mice could protect Mdr2^-/- mice from progressive liver damage. We generated double-KO (DKO: Bsep^-/- and

SUBMITTER: Wang R 

PROVIDER: S-EPMC6314265 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature