title: Mitophagy is increased during erythroid differentiation in β-thalassemia.
abstract: 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 β-thalassemia. To determine the role of mitophagy in β-thalassemia, CD34+hematopoietic progenitor cells (HPCs) were isolated from peripheral blood of β-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 β-thalassemia compared with control. Furthermore, the expression of LC3-II and Nix, as well as degradation of p62, in β-thalassemia was higher than in the control. In sum, our data suggest that selective mitophagy is enhanced during erythrocyte differentiation in β-thalassemia.
authors: Department of Pediatrics, The Second Affiliated Hospital of Sun Yat-Sen University, No. 107, Yan Jiang Road W., Guangzhou, 510120, People's Republic of China.
authors: Limei
authors: L
authors: Wu
authors: Department of Pediatrics, The Second Affiliated Hospital of Sun Yat-Sen University, No. 107, Yan Jiang Road W., Guangzhou, 510120, People's Republic of China.
authors: Wei
authors: W
authors: Xu
authors: Department of Pediatrics, The Second Affiliated Hospital of Sun Yat-Sen University, No. 107, Yan Jiang Road W., Guangzhou, 510120, People's Republic of China.
authors: Luhong
authors: L
authors: Xu
authors: Department of Pediatrics, The Second Affiliated Hospital of Sun Yat-Sen University, No. 107, Yan Jiang Road W., Guangzhou, 510120, People's Republic of China.
authors: Qian
authors: Q
authors: Kong
authors: Department of Pediatrics, The Second Affiliated Hospital of Sun Yat-Sen University, No. 107, Yan Jiang Road W., Guangzhou, 510120, People's Republic of China. Jpfang2005@163.com.
authors: Jianpei
authors: J
authors: Fang
mesh_terms: D016022:Case-Control Studies; D002454:Cell Differentiation; D002478:Cells, Cultured; D041905:Erythroid Cells / Q000166:cytology*; D006410:Hematopoiesis; D006801:Humans; D008247:Lysosomes / Q000378:metabolism; D053078:Membrane Potential, Mitochondrial; D008565:Membrane Proteins / Q000378:metabolism; D008869:Microtubule-Associated Proteins / Q000378:metabolism; D008928:Mitochondria / Q000378:metabolism; D063306:Mitophagy; D011518:Proto-Oncogene Proteins / Q000378:metabolism; D016271:Proto-Oncogene Proteins c-myc / Q000378:metabolism; D025521:Tumor Suppressor Proteins / Q000378:metabolism; D017086:beta-Thalassemia / Q000097:blood / Q000209:etiology*
chemical_list: C116838:BNIP3L protein, human; C477012:MAP1LC3A protein, human; D008565:Membrane Proteins; D008869:Microtubule-Associated Proteins; D011518:Proto-Oncogene Proteins; D016271:Proto-Oncogene Proteins c-myc; D025521:Tumor Suppressor Proteins
keywords: Hematopoiesis; Mitochondrial; Mitophagy; β-Thalassemia