Molecular basis of multiple UDP-glucuronosyltransferase isoenzyme deficiencies in the hyperbilirubinemic rat (Gunn rat).
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چکیده
منابع مشابه
Cerebellar hypoplasia in the hyperbilirubinemic Gunn rat: morphological aspects.
Gunn rats, a mutant strain of rats, suffer from autosomal recessive hyperbilirubinemia. The homozygotes (j/j) develop jaundice soon after birth and often exhibit kernicterus and cerebellar hypoplasia that are due to bilirubin. Therefore, j/j Gunn rats have been used as an animal model of bilirubin encephalopathy, as well as of neonatal hyperbilirubinemia. In this review, we discuss morphologica...
متن کاملCharacterization of the UDP-glucuronosyltransferase isoenzyme expressed in rat ovary and its regulation by gonadotropins.
Earlier studies have demonstrated that phenol UDP-glucuronosyltransferase (UGT) activity is up-regulated by pregnant mare's serum gonadotropin (PMSG) in rat ovary, but not liver. This phenomenon was investigated in more detail in the present study. Ovaries and livers of immature rats, rats synchronized with respect to their preovulatory and corpus luteal phases by treatment with PMSG, and matur...
متن کاملAdenovirus-mediated gene therapy to restore expression and functionality of multiple UDP-glucuronosyltransferase 1A enzymes in Gunn rat liver.
The Gunn rat has been a valuable model for investigating the effect of UDP-glucuronosyltransferase 1A (UGT1A) deficiencies on drug metabolism and toxicity, but it is limited in some aspects. For example, the native Gunn rat model cannot distinguish between hepatic and extrahepatic UGT1A deficiencies in toxicological mechanisms. To extend the model's utility, we investigated the use of replicati...
متن کاملBilirubin glucuronidation by intact Gunn rat fibroblasts expressing bilirubin UDP-glucuronosyltransferase.
Crigler-Najjar (CN) disease is an inherited disorder of bilirubin metabolism. The disease is caused by a deficiency of the hepatic enzyme bilirubin UDP-glucuronosyltransferase (B-UGT). Patients with CN disease have high serum levels of the toxic compound, unconjugated bilirubin. The only defect in bilirubin metabolism of CN patients is the absence of B-UGT activity. The transplantation of cells...
متن کاملTissue mRNA expression of the rat UDP-glucuronosyltransferase gene family.
UDP-Glucuronosyltransferases (UGTs) are phase II biotransformation enzymes that glucuronidate numerous endobiotic and xenobiotic substrates. Glucuronidation increases the water solubility of the substrate and facilitates renal and biliary excretion of the resulting glucuronide conjugate. UGTs have been divided into two gene families, UGT1 and UGT2. Tissue distribution of UGTs has not been thoro...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1991
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)54390-8