Chowdhury J R, Kondapalli R, Chowdhury N R
Marion Bessin Liver Research Center, Albert Einstein College of Medicine, Bronx, New York 10461.
Adv Vet Sci Comp Med. 1993;37:149-73.
Experiments in Gunn rats have provided important insights into physiological, biochemical, and molecular mechanisms of bilirubin conjugation and disposition. The Gunn rat is a natural model for bilirubin encephalopathy, and much of our knowledge of bilirubin toxicity and its treatment has come from studies performed in Gunn rats. The genetic lesion in Gunn rats closely parallel those in Crigler-Najjar syndrome, Type I. Presently, the Gunn rat model is being used to develop methods for gene therapy for inherited bilirubin-UGT deficiency. Thus, Gunn rats continue to be a valuable model for the investigation of inherited UGT deficiency and severe nonhemolytic unconjugated hyperbilirubinemia.
对冈恩大鼠进行的实验为胆红素结合与代谢的生理、生化及分子机制提供了重要见解。冈恩大鼠是胆红素脑病的天然模型，我们对胆红素毒性及其治疗的许多认识都来自于对冈恩大鼠的研究。冈恩大鼠的基因缺陷与Ⅰ型克里格勒 - 纳贾尔综合征极为相似。目前，冈恩大鼠模型正被用于开发针对遗传性胆红素 - 尿苷二磷酸葡萄糖醛酸基转移酶（UGT）缺乏症的基因治疗方法。因此，冈恩大鼠仍然是研究遗传性UGT缺乏症和严重非溶血性未结合胆红素血症的宝贵模型。
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