Disruption of the Ugt1 Locus in Mice Resembles Human Crigler-Najjar Type I Disease

• Published in: The Journal of biological chemistry Vol. 283; no. 12; pp. 7901 - 7911
• Main Authors: Nguyen, Nghia, Bonzo, Jessica A., Chen, Shujuan, Chouinard, Sarah, Kelner, Michael J., Hardiman, Gary, Bélanger, Alain, Tukey, Robert H.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 21.03.2008; American Society for Biochemistry and Molecular Biology
• Subjects: Animals; Bilirubin - blood; Bilirubin - genetics; Crigler-Najjar Syndrome - blood; Crigler-Najjar Syndrome - enzymology; Crigler-Najjar Syndrome - genetics; Crigler-Najjar Syndrome - pathology; Disease Models, Animal; Glucuronosyltransferase - genetics; Glucuronosyltransferase - metabolism; Humans; Liver - enzymology; Liver - pathology; Mice; Mice, Knockout; Quantitative Trait Loci - genetics; Skin Pigmentation - genetics

The 9 UDP-glucuronosyltranferases (UGTs) encoded by the UGT1 locus in humans are key enzymes in the metabolism of most drugs as well as endogenous substances such as bile acids, fatty acids, steroids, hormones, neurotransmitters, and bilirubin. Severe unconjugated hyperbilirubinemia in humans that s...