The expanded collagen VI family: new chains and new questions.
The expanded collagen VI family: new chains and new questions.
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扩展的胶原蛋白VI家族：新的连锁店和新问题。
DOI：
10.3109/03008207.2013.822865
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发表时间：
2013
影响因子：
2.9
通讯作者：
Hansen U
中科院分区：
文献类型：
--
作者：
Fitzgerald J;Holden P;Hansen U
胶原蛋白VI是几乎所有结缔组织（包括软骨、骨、肌腱、肌肉和角膜）的细胞外基质的组分，其中它形成丰富且结构独特的微纤维，组织成不同的超结构组件。尽管VI型胶原蛋白在组织间质基质中含量最高，并且通常与基底膜密切相关，但其确切作用尚未明确。由COL6A1、COL6A2和COL6A3基因编码的三种遗传上不同的胶原VI链，α1（VI）、α2（VI）和α3（VI），在20多年前首次被描述。它们的分子组装和在先天性肌营养不良症中的作用已被广泛表征。在2008年，描述了另外三个串联排列在人类染色体3q和小鼠染色体9上的单个基因位点上的胶原VI基因。根据胶原蛋白的命名方案，新基因被命名为COL6A4、COL6A5和COL6A6，分别编码α4（VI）、α5（VI）和α6（VI）链。本综述将侧重于三个新链的知识现状。
Collagen VI is a component of the extracellular matrix of almost all connective tissues, including cartilage, bone, tendon, muscles and cornea, where it forms abundant and structurally unique microfibrils organized into different suprastructural assemblies. The precise role of collagen VI is not clearly defined although it is most abundant in the interstitial matrix of tissues and often found in close association with basement membranes. Three genetically distinct collagen VI chains, α1(VI), α2(VI) and α3(VI), encoded by the COL6A1, COL6A2 and COL6A3 genes, were first described more than 20 years ago. Their molecular assembly and role in congenital muscular dystrophy has been broadly characterized. In 2008, three additional collagen VI genes arrayed in tandem at a single gene locus on chromosome 3q in humans, and chromosome 9 in mice, were described. Following the naming scheme for collagens the new genes were designated COL6A4, COL6A5 and COL6A6 encoding the α4(VI), α5(VI) and α6(VI) chains, respectively. This review will focus on the current state of knowledge of the three new chains.