Pre-B细胞白血病原核盒转录因子1（PBX1）是TALE（三氨基酸环扩展）家族的成员，作为一个Homeodomain转录因子（TF）发挥作用。当与其他TALE蛋白二聚化时，它可以作为先导因子通过与伴侣的相互作用提供调节序列。在脊椎动物中，PBX1在囊胚阶段表达，其在人类中的生殖细胞变异与肾脏综合征性畸形有关，对脊椎动物的造血和免疫发挥重要作用。在此，我们总结了PBX1功能和PBX1对哺乳动物肾脏肿瘤、PBX1缺失动物模型和血管的影响的现有数据。数据表明，PBX1与不同伴侣（如HOX基因）的相互作用负责胚胎间充质非正常增殖和变异，而截短变异则显示出较轻微的表型（主要是隐睾和耳聋）。尽管已经确定此类相互作用是哺乳动物中许多缺陷的原因，但某些表型变异仍需要理解。因此，需要进一步研究TALE家族。版权所有©2023 Zou, Liu, Sui和Liu。

Pre-B-cell leukemia homeobox transcription factor 1 (PBX1) is a member of the TALE (three-amino acid loop extension) family and functions as a homeodomain transcription factor (TF). When dimerized with other TALE proteins, it can act as a pioneer factor and provide regulatory sequences via interaction with partners. In vertebrates, PBX1 is expressed during the blastula stage, and its germline variations in humans are interrelated with syndromic anomalies of the kidney, which plays an important role in hematopoiesis and immunity among vertebrates. Herein, we summarize the existing data on PBX1 functions and the impact of PBX1 on renal tumors, PBX1-deficient animal models, and blood vessels in mammalian kidneys. The data indicated that the interaction of PBX1 with different partners such as the HOX genes is responsible for abnormal proliferation and variation of the embryonic mesenchyme, while truncating variants were shown to cause milder phenotypes (mostly cryptorchidism and deafness). Although such interactions have been identified to be the cause of many defects in mammals, some phenotypic variations are yet to be understood. Thus, further research on the TALE family is required.Copyright © 2023 Zou, Liu, Sui and Liu.