Morphometric Study of Cauliflower Collagen Fibrils in Ehlers-Danlos Syndrome Type I

Gérald E. Piérard , Trinh L , Claudine Piérard-Franchimont , Charles M. Lapiére
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引用次数: 24

Abstract

The abnormal cauliflower collagen fibrils present in skin of patients with Ehlers-Danlostype I were studied by electron microscopy and computerized image analysis. The size control of the few fibrils displaying a cauliflower shape is apparently lost, eventuating in an increased amount of collagen polymers constitutive of these fibrils. This situation is fundamentally different from that occuring in dermatosparaxis, an animal disease considered as a model for Ehlers-Danlos syndrome.

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ehers - danlos综合征I型花椰菜胶原原纤维形态计量学研究
采用电子显微镜和计算机图像分析方法研究了ehers - danlosti型患者皮肤中存在的异常花椰菜胶原原纤维。少数显示菜花形状的原纤维的大小控制显然失去了,最终导致构成这些原纤维的胶原聚合物数量增加。这种情况与被认为是埃勒斯-丹洛斯综合征模型的动物疾病皮肤稀疏症的情况根本不同。
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