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原纤维蛋白功能的多样性:来自牙周韧带的启示。

The Diversity of Fibrillin Functions: Lessons from the Periodontal Ligament.

作者信息

Genot Elisabeth, Al Tabosh Tala, Catros Sylvain, Alonso Florian, Le Nihouannen Damien

机构信息

BioTis, U1026, INSERM, University of Bordeaux, F-33000 Bordeaux, France.

Department of Oral Surgery, CHU Bordeaux, F-33076 Bordeaux, France.

出版信息

Cells. 2025 May 22;14(11):764. doi: 10.3390/cells14110764.

DOI:10.3390/cells14110764
PMID:40497939
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12153830/
Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/12e2/12153830/e0b50005bdd3/cells-14-00764-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/12e2/12153830/e4fa43715232/cells-14-00764-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/12e2/12153830/e0b50005bdd3/cells-14-00764-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/12e2/12153830/e4fa43715232/cells-14-00764-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/12e2/12153830/e0b50005bdd3/cells-14-00764-g001.jpg

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本文引用的文献

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Analysis of Molecular Aspects of Periodontitis as a Risk Factor for Neurodegenerative Diseases: A Single-Center 10-Year Retrospective Cohort Study.牙周炎作为神经退行性疾病危险因素的分子层面分析:一项单中心10年回顾性队列研究。
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Prodomain processing controls BMP-10 bioactivity and targeting to fibrillin-1 in latent conformation.前结构域加工控制BMP - 10的生物活性以及其在潜伏构象下与原纤维蛋白-1的靶向作用。
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The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule.
原纤蛋白微原纤维的生物力学:来自睫状小带的启示。
Cells. 2024 Dec 18;13(24):2097. doi: 10.3390/cells13242097.
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Periodontal diseases and cardiovascular diseases, diabetes, and respiratory diseases: Summary of the consensus report by the European Federation of Periodontology and WONCA Europe.牙周病与心血管病、糖尿病和呼吸道疾病:欧洲牙周病学会和世界家庭医生组织欧洲分会共识报告摘要。
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Fibrillin-1 regulates endothelial sprouting during angiogenesis.纤维连接蛋白 1 调节血管生成过程中的血管内皮发芽。
Proc Natl Acad Sci U S A. 2023 Jun 6;120(23):e2221742120. doi: 10.1073/pnas.2221742120. Epub 2023 May 30.
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Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome.马凡综合征小鼠模型肾脏的细胞外基质和血管动力学。
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Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta.从马凡综合征和非马凡综合征人主动脉中分离的原纤维蛋白-1微纤维的纳米级结构比较。
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Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory latent TGFβ-binding site.纤维连接蛋白微纤维结构确定了遗传性致病突变的长程效应,这些突变影响关键调节性潜伏 TGFβ 结合位点。
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