...
首页> 外文期刊>Journal of Cellular Physiology >Characterization of Human Dermal Fibroblasts in Fabry Disease
【24h】

Characterization of Human Dermal Fibroblasts in Fabry Disease

机译:人类皮肤成纤维细胞的特征Fabry疾病

获取原文
获取原文并翻译 | 示例
           

摘要

Fabry disease (FD) is a hereditary X-linked metabolic lysosomal storage disorder due to insufficient amounts or a complete lack of the lysosomal enzyme -galactosidase A (-GalA). The loss of -GalA activity leads to an abnormal accumulation of globotriaosylcerami (Gb3) in lysosomes and other cellular components of different tissues and cell types, affecting the cell function. However, whether these biochemical alterations also modify functional processes associated to the cell mitotic ability is still unknown. The goal of the present study was to characterize lineages of human dermal fibroblasts (HDFs) of FD patients and healthy controls focusing on Gb3 accumulation, expression of chloride channels that regulate proliferation, and proliferative activity. The biochemical and functional analyses indicate the existence of quantitative differences in some but not all the parameters of cytoskeletal organization, proliferation, and differentiation processes. J. Cell. Physiol. 230: 192-203, 2016. (c) 2015 Wiley Periodicals, Inc.
机译:Fabry疾病(FD)是一种遗传性x连锁代谢障碍由于溶酶体存储不足或完全缺乏溶酶体酶牛乳糖(晚会)。联欢晚会活动的损失会导致异常积累globotriaosylcerami (Gb3)溶酶体和其他细胞成分不同的组织和细胞类型,影响细胞的功能。改变也修改功能流程细胞有丝分裂能力仍然是有关未知的。描述人类皮肤成纤维细胞的血统(HDFs)的FD患者和健康对照组关注Gb3积累,表达氯通道调节增殖,和增殖活动。功能分析表明的存在定量差异在一些但不是全部细胞骨架组织的参数,增殖和分化过程。细胞。期刊、公司。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号