Laboratoire de Biologie Tissulaire et Ingénierie Thérapeutique (LBTI), UMR CNRS 5305, Institut de Biologie et Chimie des Protéines, Université Lyon 1, 7, Passage du Vercors, CEDEX 07, F-69367 Lyon, France.
Int J Mol Sci. 2022 Feb 14;23(4):2102. doi: 10.3390/ijms23042102.
Many extensible tissues such as skin, lungs, and blood vessels require elasticity to function properly. The recoil of elastic energy stored during a stretching phase is provided by elastic fibers, which are mostly composed of elastin and fibrillin-rich microfibrils. In arteries, the lack of elastic fibers leads to a weakening of the vessel wall with an increased risk to develop cardiovascular defects such as stenosis, aneurysms, and dissections. The development of new therapeutic molecules involves preliminary tests in animal models that recapitulate the disease and whose response to drugs should be as close as possible to that of humans. Due to its superior in vivo imaging possibilities and the broad tool kit for forward and reverse genetics, the zebrafish has become an important model organism to study human pathologies. Moreover, it is particularly adapted to large scale studies, making it an attractive model in particular for the first steps of investigations. In this review, we discuss the relevance of the zebrafish model for the study of elastic fiber-related vascular pathologies. We evidence zebrafish as a compelling alternative to conventional mouse models.
许多可伸展的组织，如皮肤、肺和血管，需要弹性才能正常运作。弹性纤维提供了在伸展阶段储存的弹性能量的回弹力，它们主要由富含弹性蛋白和原纤维蛋白的微纤维组成。在动脉中，弹性纤维的缺乏会导致血管壁变弱，增加出现心血管缺陷的风险，如狭窄、动脉瘤和夹层。新治疗分子的开发涉及在动物模型中进行初步测试，这些模型重现了疾病，并且对药物的反应应该尽可能接近人类。由于其优越的体内成像可能性和广泛的正向和反向遗传学工具包，斑马鱼已成为研究人类疾病的重要模式生物。此外，它特别适合于大规模研究，使其成为调查初始步骤的一个有吸引力的模型。在这篇综述中，我们讨论了斑马鱼模型在研究与弹性纤维相关的血管疾病中的相关性。我们证明了斑马鱼是传统小鼠模型的一个有吸引力的替代方案。
