Chung Keshi, Millet Malvina, Rouillon Ludivine, Zine Azel
LBN, Laboratory of Bioengineering and Nanoscience, University of Montpellier, 34193 Montpellier, France.
Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, MA 02114, USA.
Biomedicines. 2024 Oct 4;12(10):2262. doi: 10.3390/biomedicines12102262.
Pluripotent stem cells (PSCs) offer many potential research and clinical benefits due to their ability to differentiate into nearly every cell type in the body. They are often used as model systems to study early stages of ontogenesis to better understand key developmental pathways, as well as for drug screening. However, in order to fully realise the potential of PSCs and their translational applications, a deeper understanding of developmental pathways, especially in humans, is required. Several signalling molecules play important roles during development and are required for proper differentiation of PSCs. The concentration and timing of signal activation are important, with perturbations resulting in improper development and/or pathology. Bone morphogenetic proteins (BMPs) are one such key group of signalling molecules involved in the specification and differentiation of various cell types and tissues in the human body, including those related to tooth and otic development. In this review, we describe the role of BMP signalling and its regulation, the consequences of BMP dysregulation in disease and differentiation, and how PSCs can be used to investigate the effects of BMP modulation during development, mainly focusing on otic development. Finally, we emphasise the unique role of BMP4 in otic specification and how refined understanding of controlling its regulation could lead to the generation of more robust and reproducible human PSC-derived otic organoids for research and translational applications.
多能干细胞(PSCs)因其能够分化为体内几乎所有细胞类型而具有许多潜在的研究和临床益处。它们常被用作模型系统来研究个体发生的早期阶段,以更好地理解关键的发育途径,也用于药物筛选。然而,为了充分实现多能干细胞的潜力及其转化应用,需要更深入地了解发育途径,尤其是人类的发育途径。几种信号分子在发育过程中发挥重要作用,是多能干细胞正常分化所必需的。信号激活的浓度和时间很重要,干扰会导致发育异常和/或病理状况。骨形态发生蛋白(BMPs)就是这样一组关键的信号分子,参与人体各种细胞类型和组织的特化和分化,包括与牙齿和内耳发育相关的细胞和组织。在这篇综述中,我们描述了BMP信号传导的作用及其调控、BMP失调在疾病和分化中的后果,以及多能干细胞如何用于研究发育过程中BMP调节的影响,主要聚焦于内耳发育。最后,我们强调BMP4在内耳特化中的独特作用,以及对其调控的精细理解如何能够产生更强大、可重复的源自人类多能干细胞的内耳类器官,用于研究和转化应用。