一只患有类似于埃勒斯-当洛综合征的结缔组织缺陷的兔子的胶原纤维异常。

Abnormalities of collagen fibrils in a rabbit with a connective tissue defect similar to Ehlers-Danlos syndrome.

作者信息

Brown P J, Young R D, Cripps P J

机构信息

Comparative Pathology Laboratory, University of Bristol School of Veterinary Science, Langford.

出版信息

Res Vet Sci. 1993 Nov;55(3):346-50. doi: 10.1016/0034-5288(93)90105-o.

DOI:10.1016/0034-5288(93)90105-o

PMID:8284499

Abstract

A morphometric ultrastructural study was performed to confirm the presence of an abnormality of the collagen fibrils in a rabbit with a connective tissue defect similar to Ehlers-Danlos syndrome. Median fibril diameter and perimeter were not altered but their ranges were significantly increased. As indicated by the median fibril 'form factor', fibrils were significantly more irregular in shape; the range of irregularity in shape was also increased. Fibril periodicity was unchanged. The results are discussed in relation to collagen fibril structure and fibril abnormalities in similar diseases in man and other animals.

摘要

进行了一项形态计量超微结构研究，以证实一只患有类似于埃勒斯-当洛综合征的结缔组织缺陷的兔子中胶原纤维存在异常。纤维的中位直径和周长未改变，但范围显著增加。从中位纤维“形态因子”可以看出，纤维形状明显更不规则；形状不规则的范围也有所增加。纤维周期未改变。结合人类和其他动物类似疾病中的胶原纤维结构和纤维异常对结果进行了讨论。