The Role of Tricho-Rhino-Phalangeal Syndrome (TRPS) 1 in Apoptosis during Embryonic Development and Tumor Progression.
The Role of Tricho-Rhino-Phalangeal Syndrome (TRPS) 1 in Apoptosis during Embryonic Development and Tumor Progression.
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DOI：
10.3390/cells2030496
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发表时间：
2013-06-27
期刊：
影响因子：
6
通讯作者：
Muragaki Y
中科院分区：
文献类型：
--
作者：
Sun Y;Gui T;Shimokado A;Muragaki Y
TRPS1是一种gata型转录因子，与人鼻指骨综合征（TRPS） I型和III型密切相关，TRPS是一种常染色体显性骨骼疾病的变体。在胚胎发育过程中，Trps1抑制Sox9的表达并调节决定毛囊数量及其正常形态发生的Wnt信号通路。在生长板中，Trps1通过Gdf5信号的下游作用，靶向Pthrp、Stat3和Runx2，调控软骨细胞的凝聚、增殖、成熟和指骨关节的形成。此外，Trps1蛋白直接与Gli3的活化形式相互作用。在胚胎肾脏中，Trps1在BMP7的下游发挥作用，促进间质向上皮转化，促进小管形态发生和输尿管芽分支。此外，Trps1已被发现与前列腺癌和乳腺癌的肿瘤发生、侵袭和转移密切相关。有趣的是，在毛囊、骨骼和肾脏的发育过程中，Trps1的突变直接或通过与其他调节因子的串扰引起细胞增殖和细胞死亡的显著变化。在这篇综述中，我们将总结最近关于Trps1的研究，并试图阐明Trps1在细胞凋亡调控中的作用。
TRPS1 is a GATA-type transcription factor that is closely related to human tricho-rhino-phalangeal syndrome (TRPS) types I and III, variants of an autosomal dominant skeletal disorder. During embryonic development, Trps1 represses Sox9 expression and regulates Wnt signaling pathways that determine the number of hair follicles and their normal morphogenesis. In the growth plate, Trps1 regulates chondrocytes condensation, proliferation, and maturation and phalangeal joint formation by functioning downstream of Gdf5 signaling and by targeting at Pthrp, Stat3 and Runx2. Also, Trps1 protein directly interacts with an activated form of Gli3. In embryonic kidneys, Trps1 functions downstream of BMP7 promoting the mesenchymal-to-epithelial transition, and facilitating tubule morphogenesis and ureteric bud branching. Moreover, Trps1 has been found to be closely related to tumorigenesis, invasion, and metastasis in prostate and breast cancers. It is interesting to note that during the development of hair follicles, bones, and kidneys, mutations in Trps1 cause, either directly or through crosstalk with other regulators, a notable change in cell proliferation and cell death. In this review, we will summarize the most recent studies on Trps1 and seek to elucidate the role for Trps1 in apoptotic regulation.