Ruggiero Florence, Roulet Muriel, Bonod-Bidaud Christelle
Institut de Biologie et Chimie des Protéines, UMR CNRS 5086, IFR 128 BioSciences Lyon-Gerland, 7, passage du Vercors, 69367 Lyon cedex 07.
J Soc Biol. 2005;199(4):301-11. doi: 10.1051/jbio:2005031.
The extracellular matrix is a complex network composed of macromolecules such as collagens, proteoglycans and elastin that strongly interact with each other and with cells to maintain the structural integrity of many tissues. These interactions also sustain important cell programs such as migration, proliferation, differentiation and apoptosis. The skin, and more specifically the dermis, contains an extreme diversity of macromolecules that reflects the importance of the composition and organization of the matrix components in providing physical properties and function of the tissues. The most abundant matrix components are the collagens that form a super-family of 27 different members which are divided into different subgroups. The fibrillar collagens, types I, III and V, the FACIT collagens, types XII, XIV and XVI, and collagen VI are all expressed in the collagen-rich dermis. Although the structural features of these collagens are now well characterized, their functions remain elusive. Mutations in human collagen genes give rise to numerous connective tissue diseases including dermis disorders. For example, clinical manifestations in the classical Elhers-Danlos syndrome caused by collagen V gene mutations occur predominantly in the dermis. However, the genotype-phenotype relationship is not clearly established as well as the relation between the distribution and the function of the collagens in dermis. There is no doubt that the ongoing and future work using in vivo approaches will provide new cues regarding the function of collagens in dermis.
细胞外基质是一个由胶原蛋白、蛋白聚糖和弹性蛋白等大分子组成的复杂网络,这些大分子彼此之间以及与细胞强烈相互作用,以维持许多组织的结构完整性。这些相互作用还维持着重要的细胞程序,如迁移、增殖、分化和凋亡。皮肤,更具体地说是真皮,含有极其多样的大分子,这反映了基质成分的组成和组织在赋予组织物理特性和功能方面的重要性。最丰富的基质成分是胶原蛋白,它们形成了一个由27个不同成员组成的超家族,这些成员被分为不同的亚组。I型、III型和V型纤维状胶原蛋白、XII型、XIV型和XVI型FACIT胶原蛋白以及VI型胶原蛋白都在富含胶原蛋白的真皮中表达。尽管这些胶原蛋白的结构特征现在已经得到了很好的表征,但其功能仍然难以捉摸。人类胶原蛋白基因突变会导致多种结缔组织疾病,包括真皮疾病。例如,由V型胶原蛋白基因突变引起的经典埃勒斯-当洛综合征的临床表现主要发生在真皮中。然而,基因型-表型关系以及真皮中胶原蛋白的分布与功能之间的关系尚未明确建立。毫无疑问,正在进行的和未来使用体内方法的研究将为真皮中胶原蛋白的功能提供新的线索。