Meszaros Attila, Ahmed Junaid, Russo Giorgio, Tompa Peter, Lazar Tamas
VIB-VUB Center for Structural Biology, Vlaams Instituut voor Biotechnologie (VIB), Brussels, Belgium.
Structural Biology Brussels (SBB), Vrije Universiteit Brussel (VUB), Brussels, Belgium.
Front Med (Lausanne). 2022 Oct 20;9:1019803. doi: 10.3389/fmed.2022.1019803. eCollection 2022.
Androgen receptor (AR) is a key member of nuclear hormone receptors with the longest intrinsically disordered N-terminal domain (NTD) in its protein family. There are four mono-amino acid repeats (polyQ1, polyQ2, polyG, and polyP) located within its NTD, of which two are polymorphic (polyQ1 and polyG). The length of both polymorphic repeats shows clinically important correlations with disease, especially with cancer and neurodegenerative diseases, as shorter and longer alleles exhibit significant differences in expression, activity and solubility. Importantly, AR has also been shown to undergo condensation in the nucleus by liquid-liquid phase separation, a process highly sensitive to protein solubility and concentration. Nonetheless, in prostate cancer cells, AR variants also partition into transcriptional condensates, which have been shown to alter the expression of target gene products. In this review, we summarize current knowledge on the link between AR repeat polymorphisms and cancer types, including mechanistic explanations and models comprising the relationship between condensate formation, polyQ1 length and transcriptional activity. Moreover, we outline the evolutionary paths of these recently evolved amino acid repeats across mammalian species, and discuss new research directions with potential breakthroughs and controversies in the literature.
雄激素受体(AR)是核激素受体的关键成员,在其蛋白家族中具有最长的内在无序N端结构域(NTD)。在其NTD内有四个单氨基酸重复序列(polyQ1、polyQ2、polyG和polyP),其中两个是多态性的(polyQ1和polyG)。这两个多态性重复序列的长度与疾病,特别是与癌症和神经退行性疾病存在临床上重要的相关性,因为较短和较长的等位基因在表达、活性和溶解性方面表现出显著差异。重要的是,AR还被证明通过液-液相分离在细胞核中发生凝聚,这一过程对蛋白质的溶解性和浓度高度敏感。尽管如此,在前列腺癌细胞中,AR变体也会分配到转录凝聚物中,这已被证明会改变靶基因产物的表达。在这篇综述中,我们总结了关于AR重复序列多态性与癌症类型之间联系的当前知识,包括机制解释以及包含凝聚物形成、polyQ1长度和转录活性之间关系的模型。此外,我们概述了这些最近进化的氨基酸重复序列在哺乳动物物种中的进化路径,并讨论了文献中具有潜在突破和争议的新研究方向。