...
首页> 外文期刊>Journal of Cellular Physiology >Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice
【24h】

Impaired Hematopoiesis and Disrupted Monocyte/Macrophage Homeostasis in Mucopolysaccharidosis Type I Mice

机译:损害造血和中断单核细胞/巨噬细胞内稳态黏多糖病类型我老鼠

获取原文
获取原文并翻译 | 示例
           

摘要

Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disease caused by alpha-L-iduronidase deficiency in which heparan and dermatan sulfate degradation is compromised. Besides primary lysosomal glycosaminoglycan accumulation, further changes in cellular functions have also been described in several murine MPS models. Herein, we evaluated alterations in hematopoiesis and its implications on the production of mature progeny in a MPS I murine model. Despite the significant increase in hematopoietic stem cells, a reduction in common myeloid progenitors and granulocyte-macrophage progenitor cells was observed in Idua -/- mice bone marrow. Furthermore, no alterations in number, viability nor activation of cell death mechanisms were observed in Idua -/- mice mature macrophages but they presented higher sensitivity to apoptotic induction after staurosporine treatment. In addition, changes in Ca2+ signaling and a reduction in phagocytosis ability were also found. In summary, our results revealed significant intracellular changes in mature Idua -/- macrophages related to alterations in Idua -/- mice hematopoiesis, revealing a disruption in cell homeostasis. These results provide new insights into physiopathology of MPS I. J. Cell. Physiol. 231: 698-707, 2016. (c) 2015 Wiley Periodicals, Inc.
机译:我黏多糖病类型(MPS)是一种罕见的常染色体隐性疾病所致alpha-L-iduronidase乙酰肝素的缺乏和硫酸dermatan退化是妥协。除了初级溶酶体粘多糖进一步积累,细胞的变化也描述了在几个功能小鼠议员模型。改变造血作用及其影响在生产成熟的后代的议员小鼠模型。造血干细胞,常见的减少髓系祖细胞和granulocyte-macrophage祖细胞在Idua - / -小鼠骨髓。数,可行性和激活细胞死亡机制观察Idua - / -小鼠成熟巨噬细胞,但他们提出了更高的灵敏度staurosporine后凋亡诱导治疗。并在吞噬能力也降低发现。重要的细胞内Idua成熟的变化- / - Idua改变巨噬细胞相关- / -小鼠造血作用,揭示的中断细胞内稳态。洞察生理病理学的议员。j .细胞。杂志。231:698 - 707年,2016年。期刊、公司。

著录项

相似文献

  • 外文文献
  • 中文文献
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