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局灶节段性肾小球硬化是一种难治性慢性肾小球肾炎,属于干细胞疾病。

Focal segmental glomerular sclerosis, a type of intractable chronic glomerulonephritis, is a stem cell disorder.

作者信息

Nishimura M, Toki J, Sugiura K, Hashimoto F, Tomita T, Fujishima H, Hiramatsu Y, Nishioka N, Nagata N, Takahashi Y

机构信息

First Department of Pathology, Kansai Medical University, Osaka, Japan.

出版信息

J Exp Med. 1994 Mar 1;179(3):1053-8. doi: 10.1084/jem.179.3.1053.

Abstract

The etiopathogenesis of focal and segmental glomerular sclerosis (FGS) remains unknown. Using a new animal model for FGS (FGS mouse), we demonstrate here that bone marrow transplantation from normal mice to FGS mice with a high grade of proteinuria (+ + +) ameliorates FGS, and that the transplantation of bone marrow cells or purified hemopoietic stem cells (HSCs) from FGS mice induces FGS in normal mice. These findings strongly suggest that FGS is a stem cell disorder; the abnormalities may be genetically programmed at the level of HSCs.

摘要

局灶节段性肾小球硬化(FGS)的发病机制尚不清楚。我们利用一种新的FGS动物模型(FGS小鼠),在此证明将正常小鼠的骨髓移植到具有高度蛋白尿(+++)的FGS小鼠可改善FGS,并且将FGS小鼠的骨髓细胞或纯化的造血干细胞(HSC)移植到正常小鼠中会诱发FGS。这些发现强烈表明FGS是一种干细胞疾病;异常可能在造血干细胞水平上是由基因编程的。

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