Genot Elisabeth, Al Tabosh Tala, Catros Sylvain, Alonso Florian, Le Nihouannen Damien
BioTis, U1026, INSERM, University of Bordeaux, F-33000 Bordeaux, France.
Department of Oral Surgery, CHU Bordeaux, F-33076 Bordeaux, France.
Cells. 2025 May 22;14(11):764. doi: 10.3390/cells14110764.
Marfan syndrome is caused by a mutation in the gene encoding fibrillin-1. This extracellular matrix glycoprotein, which assembles into microfibrils, is best known for its scaffolding role in the production of elastic fibers responsible for connective tissue elasticity and tensile strength. Research into Marfan syndrome mainly focuses on the pathophysiology involved in the degeneration of elastin-rich elastic fibers, which are essential components of the aortic wall. However, fibrillin-1 also exists in elastin-poor (elaunin) or elastin-free (oxytalan) microfibril bundles that were first described in the periodontal ligament (PDL). This dynamic, densely cellular, and highly vascularized tissue anchors teeth in their bone sockets and acts as a protective shock absorber during chewing. Current knowledge suggests that fibrillin microfibrils mechanically support blood vessels in the PDL and ensure their proper functioning. However, many more insights on the roles of fibrillin, especially independently of elastin, can be extracted from this tissue. Here, we review the phenotypic and functional characteristics of the PDL in connection with fibrillin-1, focusing on those related to microvessels. This review aims to shed light on this often-overlooked fibrillin-rich resource as a model for future studies investigating fibrillin functions in health and Marfan disease.
马凡综合征由编码原纤蛋白-1的基因突变引起。这种细胞外基质糖蛋白组装成微原纤维,因其在产生负责结缔组织弹性和抗张强度的弹性纤维过程中的支架作用而最为人所知。对马凡综合征的研究主要集中在富含弹性蛋白的弹性纤维变性所涉及的病理生理学上,这些弹性纤维是主动脉壁的重要组成部分。然而,原纤蛋白-1也存在于牙周韧带(PDL)中首次描述的弹性蛋白含量低(弹力素)或不含弹性蛋白(氧化弹力纤维)的微原纤维束中。这种动态、细胞密集且血管高度丰富的组织将牙齿固定在牙槽窝中,并在咀嚼过程中起到保护性减震器的作用。目前的知识表明,原纤蛋白微原纤维在机械上支持PDL中的血管并确保其正常功能。然而,从这个组织中可以提取出更多关于原纤蛋白作用的见解,尤其是独立于弹性蛋白的作用。在这里,我们结合原纤蛋白-1综述PDL的表型和功能特征,重点关注与微血管相关的特征。本综述旨在阐明这个经常被忽视的富含原纤蛋白的资源,作为未来研究原纤蛋白在健康和马凡病中功能的模型。