FHL(four-and-a-half-LIM domain)是含4(1/2)个LIM结构域富含半胱氨酸的细胞骨架蛋白,LIM是一种在C.elegans线虫的Lin-1和Mec-3基因及大鼠Isl-1基因编码的DNA结合蛋白中分离鉴定出来的基因序列,LIM取三个基因的首字母而成。FHL家族含FHL-1-5五个成员,而FHL-1-3最早发现在心脏的发育过程中起重要作用,后面发现在肺动脉高压中有促进增殖、迁移等作用。本文就FHL家族和肺动脉高压关系作一综述,阐明FHL蛋白在PH进程中的重要作用。
FHL(four-and-a-half-LIM domain) a cytoskeletal protein which containing 4(1/2) LIM domain and cysteine-rich, The name of the LIM domain, is derived from three homeodomain proteins(Caenorhabditis elegans Lin-11, rat Isl-1, and C. elegans Mec-3). This domain has been recognized in a variety of cytoplasmic and nuclear functional molecules. FHL family including FHL-1-5 five members, and FHL-1-3 was discovered important effect in the development of the heart, the latter found that they can promote proliferation, migration and so on in the process of PH. This review aims to summarize the relathionship between FHL and PH.