- 作者: Huey-Ling Chen; Renxue Wang; Hui-Ling Chen; Wuh-Liang Hwu; Yung-Ming Jeng; Mei-Hwei Chang; Victor Ling
- 作者服務機構: Department of Pediatrics, National Taiwan University College ofMedicine and Hospital, Taipei, Taiwan, ROC; Department of Primary Care Medicine, National TaiwanUniversity College of Medicine and Hospital,Taipei, Taiwan, ROC
- 中文摘要: --
- 英文摘要: Cell transplantation is a potential therapy foracquired or inherited liver diseases. Donor-derived hepatocytes(DDH) have been found in humans and mice afterbone marrow transplantation (BMT) but with highly variablefrequencies in different disease models. To test theeffect of liver repopulation after BMT in inherited cholestaticliver diseases, spgp (sister of P-glycoprotein, or bilesalt export pump, abcb11) knockout mice, a model forhuman progressive intrahepatic cholestasis type 2 withdefects in excreting bile salts across the hepatocyte canalicularmembrane, were transplanted with bone marrowcells from enhanced green fluorescent protein (EGFP)transgenic donor mice after lethal irradiation. One to6 months later, scattered EGFP-positive DDHs with positivespgp staining were observed in the liver. Thesehepatocytes had been incorporated into hepatic plates andstained positively with hepatocyte-specific marker albumin.RT-PCR for the spgp gene revealed positiveexpression in the liver of sgsp knockout mice that hadreceived the transplant. Bile acid analysis of bile samplesshowed that these mice also had higher levels of totalbiliary bile acid and taurocholic acid concentration thanknockout mice without transplantation, indicating thatBMT partially improved biliary bile acid secretion. Ourresults indicate that bone marrow cells could serve as apotential source for restoration of hepatic functions inchronic metabolic liver disease.
- 中文關鍵字: --
- 英文關鍵字: abcb11; spgp; Progressive familial intrahepatic cholestasis; Bile salt export pump; Cell transplantation; Cholestasis; Bile acid metabolism