系统性透明变性或青少年透明纤维瘤病。培养的皮肤成纤维细胞的超微结构和生化研究。
Systemic hyalinosis or juvenile hyaline fibromatosis. Ultrastructural and biochemical study of cultured skin fibroblasts.
作者信息
Iwata S, Horiuchi R, Maeda H, Ishikawa H
出版信息
Arch Dermatol Res. 1980;267(2):115-21. doi: 10.1007/BF00569097.
PMID:6157366
Abstract
Biochemical examinations of a hyalinized skin tumor of systemic hyalinosis (juvenile hyaline fibromatosis) revealed an increase in chrondroitin sulfate synthesis by skin fibroblasts with an enlarged, rough surfaced endoplasmic reticulum as well as an increase in chondroitin sulfate content. Thse results indicate that systemic hyalinosis is a connective tissue disorder characterized by abnormal GAG synthesis of fibroblasts.
摘要
对系统性透明变性（青少年透明纤维瘤病）的透明化皮肤肿瘤进行的生化检查显示，皮肤成纤维细胞的硫酸软骨素合成增加，内质网增大且表面粗糙，同时硫酸软骨素含量也增加。这些结果表明，系统性透明变性是一种以成纤维细胞异常合成糖胺聚糖为特征的结缔组织疾病。
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