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首页> 外文期刊>The biochemical journal >Characterization of a type VI collagen-related Mr-140 000 protein from cutis-laxa fibroblasts in culture
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Characterization of a type VI collagen-related Mr-140 000 protein from cutis-laxa fibroblasts in culture

机译：来自培养的Cutis-laxa成纤维细胞的VI型胶原相关MR-140 000蛋白的表征

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pThe precise biochemical defects in connective-tissue metabolism that are responsible for the laxity of skin seen in the syndrome of cutis laxa are largely unknown. We have studied fibroblasts cultured from skin explants of a 2-year-old male with the syndrome. Electron-microscopic examination of this skin revealed decreased amounts of amorphous elastin and an increase in elastin-associated microfibrils. Although the cultured fibroblasts were similar to control skin fibroblasts in morphology, growth rate and total protein synthesis, there was a 4-6-fold increase in accumulation of a collagenous protein of Mr 140 000 in both the culture medium and in the cell layer. This protein was structurally distinct from collagen types I, III, IV, V and VIII. It was found to be related to a cell-surface-associated glycoprotein, GP140, by both antigenic cross-reactivity and peptide mapping. Our data support observations that GP140 is a precursor of at least one form of pepsin-extracted type VI collagen./p
机译：>在Cutis Laxa综合症中观察到的皮肤的松弛的结缔组织代谢中的精确生化缺陷在很大程度上是未知的。我们用综合症学习了一名2岁男性的皮肤外植体培养的成纤维细胞。这种皮肤的电子显微镜检查显示出的无定形弹性蛋白的量减少，并且增加了弹性蛋白相关的微纤维。虽然培养的成纤维细胞类似于对形态，生长速率和总蛋白质合成的对皮肤成纤维细胞相似，但在培养基和细胞层中，MR 140000的胶原蛋白的积累增加4-6倍。该蛋白质在结构上与胶原蛋白类型I，III，IV，V和VIII不同。发现它与细胞表面相关的糖蛋白GP140有关，通过抗原交叉反应性和肽测绘。我们的数据支持观察到GP140是至少一种形式的胃蛋白酶提取的VI胶原蛋白的前体。

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