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胶原疾病与胶原的生物合成

Collagen diseases and the biosynthesis of collagen.

作者信息

Prockop D J, Guzman N A

出版信息

Hosp Pract. 1977 Dec;12(12):61-8. doi: 10.1080/21548331.1977.11707242.

Abstract

Increasing knowledge concerning the molecular structure of collagen and the steps involved in its biosynthesis provides the basis for a new look at the collagen diseases. Some, like the Ehlers-Danlos syndrome, are now known to be the expression of primary molecular defects; others, such as scurvy and scleroderma, appear to be secondary to some process that disrupts normal controls over collagen synthesis or deposition.

摘要

对胶原蛋白分子结构及其生物合成步骤的了解不断增加,为重新审视胶原蛋白疾病提供了依据。现在已知一些疾病,如埃勒斯-丹洛斯综合征,是原发性分子缺陷的表现;而其他一些疾病,如坏血病和硬皮病,似乎是某些破坏胶原蛋白合成或沉积正常调控过程的继发结果。

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