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Real Time Monitoring of Intracellular Bile Acid Dynamics Using a Genetically Encoded FRET-based Bile Acid Sensor
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Inborn Errors of Bile Acid Metabolism

James E Heubi1, Kenneth D R Setchell1, Kevin E Bove1

  • 1Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 240 Sabin Way, Cincinnati, OH 45229, USA; Division of Pathology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 240 Sabin Way, Cincinnati, OH 45229, USA.

Clinics in Liver Disease
|September 30, 2018
PubMed
Summary

Inborn errors of bile acid metabolism cause neonatal cholestasis and liver disease. Diagnosis involves testing for hyperbilirubinemia with normal bile acids, and cholic acid treats many defects.

Keywords:
CirrhosisLiverNeonatal cholestasisZellweger spectrum disorder

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