Focal dermal hypoplasia (Goltz syndrome): a decreased accumulation of hyaluronic acid in three-dimensional culture.
Focal dermal hypoplasia (Goltz syndrome): a decreased accumulation of hyaluronic acid in three-dimensional culture.
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局灶性真皮发育不全（Goltz 综合征）：三维培养中透明质酸积累减少。
DOI：
10.2340/0001555576365367
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发表时间：
1996
影响因子：
3.6
通讯作者：
Y. Miyachi
中科院分区：
文献类型：
--
作者：
M. Sato;O. Ishikawa;Y. Yokoyama;A. Kondo;Y. Miyachi
我们报告一位28岁的女性患有局灶性皮肤发育不全（Goltz综合征）。我们比较了受影响和未受影响皮肤的成纤维细胞的生长动力学和I型胶原和糖胺聚糖的产生。成纤维细胞生长在常规培养基中补充抗坏血酸2-磷酸，使成纤维细胞从体外组织样结构。患侧皮肤成纤维细胞群体倍增时间略短于健侧皮肤。受影响的皮肤和未受影响的皮肤之间释放到媒体的I型前胶原的羧基末端前肽的水平没有差异。然而，受影响的皮肤成纤维细胞的细胞层揭示了透明质酸衍生的二糖单位（Δ Di-HA）的量减少。局部真皮发育不良的皮肤改变可能与受累真皮中糖胺聚糖代谢异常有关。
We report a 28-year-old female with focal dermal hypoplasia, (Goltz syndrome). We compared the growth kinetics and the production of type I collagen and glycosaminoglycans by fibroblasts from affected and unaffected skin. Fibroblasts were grown in conventional medium supplemented with ascorbic acid 2-phosphate, which makes fibroblasts from a tissue-like structure in vitro. The population doubling time of fibroblasts in affected skin was slightly shorter than that of unaffected skin. There was no difference in the levels of the carboxyterminal propeptide of type I procollagen liberated into the media between affected skin and unaffected skin. However, the cell layer of affected skin fibroblasts revealed a decreased amount of hyaluronic acid-derived disaccharide unit (delta Di-HA). The abnormal metabolism of glycosaminoglycan in the affected dermis might be involved in the development of skin changes in focal dermal hypoplasia.
局灶性真皮发育不全：培养物中皮肤成纤维细胞的异常生长特征。
DOI：
10.1111/1523-1747.ep12522586
发表时间：
1980
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Uitto,J;Bauer,EA;Santa-Cruz,DJ;Loewinger,RJ;Eisen,AZ
通讯作者：
Eisen,AZ