Gunn rats as a surrogate model for evaluation of hepatocyte transplantation-based therapies of crigler-najjar syndrome type 1

Zsuzsanna Polgar, Yanfeng Li, Xia Li Wang, Chandan Guha, Namita Roy-Chowdhury, Jayanta Roy-Chowdhury

Research output: Chapter in Book/Report/Conference proceedingChapter

4 Scopus citations

Abstract

Liver transplantation has been established as a curative therapy for acute and chronic liver failure, as well as liver-based inherited metabolic diseases. Because of the complexity of organ transplantation and the worldwide shortage of donor organs, hepatocyte transplantation is being developed as a bridging therapy until donor organs become available, or for amelioration of inherited liver-based diseases. The Gunn rat is a molecular and metabolic model of Crigler-Najjar syndrome type 1, which is characterized by lifelong unconjugated hyperbilirubinemia due to the lack of uridinediphosphoglucuronate glucuronosyltransferase-1 (UGT1A1)-mediated bilirubin glucuronidation. Gunn rats are convenient for evaluating the effect of hepatocyte transplantation or gene therapy, because the extent of UGT1A1 replacement can be assessed by serial determination of serum bilirubin levels, and excretion of bilirubin glucuronides in bile provide definitive evidence of the function of the transplanted hepatocytes or the effect of gene therapy. The core techniques involved in hepatocyte transplantation in Gunn rats are discussed in this chapter.

Original languageEnglish (US)
Title of host publicationMethods in Molecular Biology
PublisherHumana Press Inc.
Pages131-147
Number of pages17
Volume1506
DOIs
StatePublished - 2017

Publication series

NameMethods in Molecular Biology
Volume1506
ISSN (Print)10643745

Keywords

  • Bile pigment analysis
  • Bilirubin
  • Crigler-najjar syndrome
  • Gunn rats
  • Hepatocyte transplantation

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics

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    Polgar, Z., Li, Y., Li Wang, X., Guha, C., Roy-Chowdhury, N., & Roy-Chowdhury, J. (2017). Gunn rats as a surrogate model for evaluation of hepatocyte transplantation-based therapies of crigler-najjar syndrome type 1. In Methods in Molecular Biology (Vol. 1506, pp. 131-147). (Methods in Molecular Biology; Vol. 1506). Humana Press Inc.. https://doi.org/10.1007/978-1-4939-6506-9_9