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胶原和糖蛋白使结缔组织具有抵抗和传达机械力的能力。以往认为胶原仅仅是惰性的,其作用长期被忽视。近来对胶原生物合成和降解的复杂途径的研究迅速发展,同时不断发现新的生物功能,包括胶原在组织形态发生中的作用,在止血中的作用及在神经肌连接点成熟中的作用等。通过对人及动物结缔组织疾病的研究促进了人们对胶原研究的兴趣。本文将论述人们较生疏的和相当少见的胶原代谢异常及疾病,并着重人类遗传性胶原代谢疾病。
Collagen and glycoproteins give connective tissue the ability to resist and transmit mechanical forces. In the past, collagen was considered inert, and its role has long been neglected. Recent studies on the complex pathways of collagen biosynthesis and degradation have developed rapidly, and new biological functions have been continuously discovered, including the role of collagen in tissue morphogenesis, the role in hemostasis, and the role in the maturation of neuromuscular junctions. Research on human and animal connective tissue diseases has stimulated interest in collagen research. This article will discuss people’s unfamiliar and relatively rare abnormal collagen metabolism and diseases, and focus on human hereditary collagen metabolism diseases.