Mitophagy is increased during erythroid differentiation in β-thalassemia
Mitophagy is increased during erythroid differentiation in β-thalassemia
复制标题
β-地中海贫血的红细胞分化过程中线粒体自噬增加
DOI：
10.1007/s12185-016-2114-z
复制
发表时间：
2017-02-01
影响因子：
2.1
通讯作者：
Fang, Jianpei
中科院分区：
文献类型：
--
作者：
Wu, Limei;Xu, Wei;Fang, Jianpei
线粒体自噬是线粒体的选择性降解，在造血过程中也起着至关重要的作用。然而，目前尚不清楚这个过程在-地中海贫血的发病机制中起什么作用，如果有的话。为了确定线粒体自噬在β -地中海贫血中的作用，我们从β -地中海贫血患者和健康对照者的外周血中分离CD34（+）造血祖细胞（HPCs）并将其分化为红细胞。我们发现，与对照组相比，β -地中海贫血患者线粒体膜去极化率显著增加，线粒体与溶酶体共定位水平更高。此外，LC3-II和Nix的表达以及p62的降解在β -地中海贫血中高于对照组。总之，我们的数据表明，在β -地中海贫血的红细胞分化过程中，选择性线粒体自噬增强。
Mitophagy is a selective degradation of mitochondria, which also plays a critical role in hematopoiesis. However, it is unclear what role, if any, this process plays in the pathogenesis of beta-thalassemia. To determine the role of mitophagy in beta-thalassemia, CD34(+) hematopoietic progenitor cells (HPCs) were isolated from peripheral blood of beta-thalassemia patients and healthy controls and differentiated into erythrocytes. We found that the ratio of mitochondrial membrane depolarization was significantly increased, and that mitochondria co-localize with lysosomes at a higher level in beta-thalassemia compared with control. Furthermore, the expression of LC3-II and Nix, as well as degradation of p62, in beta-thalassemia was higher than in the control. In sum, our data suggest that selective mitophagy is enhanced during erythrocyte differentiation in beta-thalassemia.