Extracellular matrix is a very complex assemblage of specialized molecules including collagen, elastin, proteoglycans, and attachment glycoproteins such as fibronectin and laminin. Collagen is an unusual protein because it requires multiple processing steps both within and outside the cell beginning with transcription and refinement of its messenger RNA, translation of the mature mRNA, extensive post-translational modifications, and further alterations in the extracellular compartments. If errors are made at any one of the multiple steps in this complex pathway, a defective molecule will be formed that could result in connective tissue abnormalities. Similarly, alterations in other components of the extracellular matrix can also result in clinical pathologies. Some of these clinical phenotypes have been classified as Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, and epidermolysis bullosa. This article reviews these various connective tissue disorders, describes the underlying biochemical defects, and describes their impact on tissue repair.

译文

:细胞外基质是由特殊分子组成的非常复杂的集合体,包括胶原蛋白,弹性蛋白,蛋白聚糖和附着糖蛋白(如纤连蛋白和层粘连蛋白)。胶原蛋白是一种不寻常的蛋白质,因为它需要在细胞内外进行多个处理步骤,从其信使RNA的转录和精制,成熟mRNA的翻译,广泛的翻译后修饰以及细胞外区室的进一步改变开始。如果在此复杂路径的多个步骤中的任何一个步骤上出错,就会形成有缺陷的分子,从而可能导致结缔组织异常。类似地,细胞外基质其他成分的改变也可导致临床病理。这些临床表型中的一些已被分类为Ehlers-Danlos综合征,Marfan综合征,成骨不全症和大疱性表皮松解。本文回顾了这些各种结缔组织疾病,描述了潜在的生化缺陷,并描述了它们对组织修复的影响。

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