WT1 and kidney progenitor cells

WT1 and kidney progenitor cells
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DOI:
10.4161/org.6.2.11928
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发表时间:
2010-04-01
期刊:
影响因子:
2.3
通讯作者:
Kreidberg, Jordan A.
Kreidberg, Jordan A.
中科院分区:
工程技术4区
文献类型:
--
作者:
Kreidberg, Jordan A.

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在过去的60年里,肾脏发育一直被作为器官发生过程中胚胎诱导的模型进行研究。Wilms' tumor-1(WT 1)编码转录因子和RNA结合蛋白,是最早发现的肿瘤抑制基因之一,此后不久被证明与儿童肾病和性腺发育不全的综合征形式有关。由WT 1基因无效突变引起的肾脏发育不全是由基因靶向实验引起的器官发育不全的第一个例子之一。因此,WT 1基因及其编码蛋白的研究一直处于发育生物学、肿瘤生物学和疾病分子基础的前沿。现在已知WT 1在发育过程中对肾祖细胞具有重要作用。本文将讨论我们对肾祖细胞的认识,以及这些细胞中WT 1靶基因的最新鉴定进展。
Kidney development has been studied over the past sixty years as a model of embryonic induction during organogenesis. Wilms' tumor-1 (WT1), that encodes a transcription factor and RNA-binding protein, was one of the first tumor suppressor genes identified, and was soon thereafter shown to be associated with syndromic forms of childhood kidney disease and gonadal dysgenesis. Kidney agenesis, resulting from a null mutation in the WT1 gene, was one of the first examples of organ agenesis resulting from a gene targeting experiment. Thus, the study of the WT1 gene and its encoded proteins has been at the forefront of developmental biology, tumor biology and the molecular basis for disease. WT1 is now known to have an important role in kidney progenitor cells during development. This review will discuss recent advances in our understanding of kidney progenitor cells, and the recent identification of WT1 target genes in these cells.