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常染色体隐性遗传性结合胆红素血症突变大鼠的有机阴离子转运研究

Organic anion transport study in mutant rats with autosomal recessive conjugated hyperbilirubinemia.

作者信息

Kurisu H, Kamisaka K, Koyo T, Yamasuge S, Igarashi H, Maezawa H, Uesugi T, Tagaya O

机构信息

Third Department of Internal Medicine, Tokyo Medical and Dental University, Japan.

出版信息

Life Sci. 1991;49(14):1003-11. doi: 10.1016/0024-3205(91)90301-q.

Abstract

The EHBR is a mutant rat strain with congenital conjugated hyperbilirubinemia bred from a Sprague-Dawley rat. Transport of conjugated bilirubin, indocyanine green, and tetrabromosulfophtalein from liver to bile is severely impaired in these rats. Serum bilirubin amounts to 6.0 +/- 0.05 mg/dl (n = 4) in adult rats, with 97% conjugates. The bile flow is reduced to about 65% of the control group, whereas total bile acid in 10-min bile samples is similar. Liver histology of 10 week-old rats revealed neither intracellular pigmentation nor architectural abnormalities.

摘要

EHBR是一种从斯普拉格-道利大鼠培育而来的患有先天性结合胆红素血症的突变大鼠品系。这些大鼠中,结合胆红素、吲哚菁绿和四溴酚酞磺酸钠从肝脏到胆汁的转运严重受损。成年大鼠血清胆红素含量为6.0±0.05mg/dl(n = 4),其中97%为结合物。胆汁流量降至对照组的约65%,而10分钟胆汁样本中的总胆汁酸含量相似。10周龄大鼠的肝脏组织学检查未发现细胞内色素沉着或结构异常。

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