DERMATOSPARAXIS IN A HIMALAYAN CAT .2. ULTRASTRUCTURAL STUDIES OF DERMAL COLLAGEN
DERMATOSPARAXIS IN A HIMALAYAN CAT .2. ULTRASTRUCTURAL STUDIES OF DERMAL COLLAGEN
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DOI：
10.1111/1523-1747.ep12520000
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发表时间：
1980-01-01
影响因子：
6.5
通讯作者：
HEGREBERG, GA
中科院分区：
文献类型：
--
作者：
HOLBROOK, KA;BYERS, PH;HEGREBERG, GA
皮肤松解症是一种结缔组织疾病，主要发生在绵羊和牛身上，由氨基末端前胶原肽酶活性不足引起。它的特点是脆弱，松散的皮肤，很容易撕裂轻微的创伤。一只猫被鉴定为这种前胶原肽酶有缺陷，只影响一小部分胶原分子;大部分胶原蛋白被正常加工。如透射电子显微镜和扫描电子显微镜所见，异常胶原分子的这一群体显著改变了单个胶原原纤维的结构、原纤维组装成纤维束以及纤维束整合成皮肤网状真皮中的正常编织网络。尽管临床发现不如酶缺陷更完全的绵羊和牛严重，但超微结构异常明显，并证明少数异常胶原分子可对结缔组织的结构和功能产生重大影响。
Dermatosparaxis is a connective tissue disease, primarily of sheep and cattle, that results from deficient activity of the NH2-terminal procollagen peptidase. It is characterized by fragile, loose skin that is easily torn with minor trauma. A cat was identified with a defect in this procollagen peptidase which affects only a small proportion of the collagen molecules; the majority of the collagen is processed normally. As seen by transmission electron microscopy and scanning electron microscopy, this population of aberrant collagen molecules significantly alters the structure of individual collagen fibrils, the assembly of fibrils into fiber bundles and the integration of fiber bundles into a normal, woven network in the reticular dermis of skin. Although the clinical finding are less severe than those in sheep and cattle where the enzymatic defect is more complete the ultrastuctural abnormalities are marked and demonstrate that a minority of abnormal collagen molecules can have a major effect on the structure and function of connective tissues.