Yoshida Y, Tokusashi Y, Lee G H, Ogawa K
Department of Pathology, Asahikawa Medical College, Japan.
Gastroenterology. 1996 Dec;111(6):1654-60. doi: 10.1016/s0016-5085(96)70029-x.
BACKGROUND & AIMS: Long-Evans cinnamon (LEC) rats are an animal model of Wilson's disease. This study investigated whether intrahepatic transplantation of normal hepatocytes can prevent Wilson's disease in LEC rats.
Hepatocytes of newborn Long-Evans agouti (LEA) rats were transplanted into the livers of LEC rats before onset of hepatic disease. Expression of normal transcripts from the gene linked to Wilson's disease was investigated at 30 weeks of age by reverse-transcription polymerase chain reaction and in situ hybridization.
Eight of 16 (50.0%) untreated LEC rats died of hepatic failure during 20-30 weeks of age. Of the 27 LEC rats with transplanted cells, 2 (7.4%) died of hepatic failure and 4 died of ileus complicated by the surgical treatment. In the recipient livers, the transplanted cells comprised 4%-20% of the hepatocyte populations, expressing normal messenger RNA transcribed from the Wilson's disease gene, and hepatic copper deposition was reduced to approximately 60% of that in untreated LEC rats.
Transplantation of normal hepatocytes prevents Wilson's disease in LEC rats.
长 Evans 肉桂色（LEC）大鼠是威尔逊病的动物模型。本研究调查了正常肝细胞肝内移植是否能预防 LEC 大鼠的威尔逊病。
在肝病发作前，将新生的长 Evans 刺鼠（LEA）大鼠的肝细胞移植到 LEC 大鼠的肝脏中。通过逆转录聚合酶链反应和原位杂交，在 30 周龄时研究与威尔逊病相关基因的正常转录本的表达。
16 只未治疗的 LEC 大鼠中有 8 只（50.0%）在 20 - 30 周龄时死于肝衰竭。在 27 只接受细胞移植的 LEC 大鼠中，2 只（7.4%）死于肝衰竭，4 只死于手术治疗并发的肠梗阻。在受体肝脏中，移植细胞占肝细胞群体的 4% - 20%，表达从威尔逊病基因转录的正常信使核糖核酸，肝铜沉积减少至未治疗的 LEC 大鼠的约 60%。
正常肝细胞移植可预防 LEC 大鼠的威尔逊病。
