Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils.
Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils.
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纤维蛋白基因的发育表达表明细胞外微纤维的异质性。
DOI：
10.1083/jcb.129.4.1165
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发表时间：
1995-05
期刊：
影响因子：
--
通讯作者：
Ramirez F
中科院分区：
文献类型：
--
作者：
Zhang H;Hu W;Ramirez F
细胞外微纤维，单独或与弹性蛋白结合，赋予各种结缔组织关键的生物力学特性。关于微纤维的组成或负责其空间组织成组织特异性大聚集体的因素知之甚少。最近的工作揭示了两种结构相关的微纤维组分的存在，称为微纤维蛋白-1和微纤维蛋白-2。这些糖蛋白之间以及它们与微纤丝和弹性纤维的其他组分之间的功能关系尚不清楚。作为阐明这些重要观点的第一步，我们比较了哺乳动物胚胎发生过程中的peptide基因的表达模式。结果表明，这两个基因的差异表达，在发育阶段和组织分布。在大多数情况下，与repeat in-1转录物相比，repeat in-2转录物出现得更早，积累的时间更短。合成的β-淀粉样蛋白-1相关的晚期形态发生和外观的明确的器官结构; β-淀粉样蛋白-2的合成，另一方面，与早期形态发生，特别是与弹性的开始。这些发现间接支持了我们最初的假设，即原纤维蛋白有助于微纤维的组成和功能异质性。现有的证据也与原纤维蛋白在微纤维生理学中可能具有不同但相关的作用的概念一致。因此，我们建议，主要是力轴承的结构支持，而主要是调节弹性纤维组装的早期过程中，repeat in-2。
Extracellular microfibrils, alone or in association with elastin, confer critical biomechanical properties on a variety of connective tissues. Little is known about the composition of the microfibrils or the factors responsible for their spatial organization into tissue- specific macroaggregates. Recent work has revealed the existence of two structurally related microfibrillar components, termed fibrillin-1 and fibrillin-2. The functional relationships between these glycoproteins and between them and other components of the microfibrils and elastic fibers are obscure. As a first step toward elucidating these important points, we compared the expression pattern of the fibrillin genes during mammalian embryogenesis. The results revealed that the two genes are differentially expressed, in terms of both developmental stages and tissue distribution. In the majority of cases, fibrillin-2 transcripts appear earlier and accumulate for a shorter period of time than fibrillin-1 transcripts. Synthesis of fibrillin-1 correlates with late morphogenesis and the appearance of well-defined organ structures; fibrillin-2 synthesis, on the other hand, coincides with early morphogenesis and, in particular, with the beginning of elastogenesis. The findings lend indirect support to our original hypothesis stating that fibrillins contribute to the compositional and functional heterogeneity of the microfibrils. The available evidence is also consistent with the notion that the fibrillins might have distinct, but related roles in microfibril physiology. Accordingly, we propose that fibrillin-1 provides mostly force-bearing structural support, whereas fibrillin-2 predominantly regulates the early process of elastic fiber assembly.