Collagenase expression in skin fibroblasts from families with recessive dystrophic epidermolysis bullosa.
Collagenase expression in skin fibroblasts from families with recessive dystrophic epidermolysis bullosa.
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隐性营养不良性大疱性表皮松解症家族皮肤成纤维细胞中胶原酶的表达。
DOI：
10.1111/1523-1747.ep13071274
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发表时间：
1989
期刊：
影响因子：
--
通讯作者：
Bauer,EA
中科院分区：
文献类型：
--
作者：
Winberg,JO;Gedde-DahlJr,T;Bauer,EA
对显性(D-EBD)和隐性(R-EBD)大疱性营养不良表皮松解症斯堪的纳维亚家系皮肤成纤维细胞的胶原酶产生进行了研究。由于同一受试者从好发部位获得的成纤维细胞与从非好发部位获得的成纤维细胞产生相同数量的免疫反应胶原酶，因此排除了由于身体部位来源的异质性。体外胶原酶的产生在个体和家庭之间有很大的差异。在R-EBD组中，与他们的健康亲属、其他EB类型和对照组相比，18名患者中有4名在体外显示出免疫反应胶原酶水平升高。这表明，体外胶原酶产量升高并不是整个疾病组的标志，这种被称为R-EBD的疾病在病因和病理上可能是不同的。
The collagenase production of cultured skin fibroblasts from Scandinavian families with dominant (D-EBD) and recessive (R-EBD) epidermolysis bullosa dystrophica has been investigated. Heterogeneity as a result of body location origin has been ruled out as fibroblasts obtained from predilection sites produce the same amount of immunoreactive collagenase as those obtained from non-predilection sites of the same subjects. Large variations in in vitro collagenase production were found between individuals and families. Within the R-EBD group, four out of eighteen patients showed an in vitro elevated level of immunoreactive collagenase compared to their healthy relatives, other EB types, and the control group. This shows that an in vitro elevated collagenase production is not a marker for the entire disease group and that the disease denoted as R-EBD probably is etiologically and pathogenetically heterogeneous.