Bilirubin‐UDP‐glucuronosyltransferase: Genetic defect of the hyperbilirubinemic gunn rat, a model of crigler‐najjar syndrome type I
نویسندگان
چکیده
منابع مشابه
the investigation of the relationship between type a and type b personalities and quality of translation
چکیده ندارد.
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...
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Correction of the UDP-glucuronosyltransferase gene defect in the gunn rat model of crigler-najjar syndrome type I with a chimeric oligonucleotide.
Crigler-Najjar syndrome type I is characterized by unconjugated hyperbilirubinemia resulting from an autosomal recessive inherited deficiency of hepatic UDP-glucuronosyltransferase (UGT) 1A1 activity. The enzyme is essential for glucuronidation and biliary excretion of bilirubin, and its absence can be fatal. The Gunn rat is an excellent animal model of this disease, exhibiting a single guanosi...
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ژورنال
عنوان ژورنال: Stem Cells
سال: 1994
ISSN: 1066-5099,1549-4918
DOI: 10.1002/stem.5530120714