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Single-cell profiling of ineffective erythropoiesis in a mouse model of ß-thalassaemia intermedia.
Peng, Yuanliang; Liang, Long; Zhang, Haihang; Liu, Hong; Zhang, Guanxiong; Sun, Shuming; Guo, Xianfeng; Wang, Yanpeng; Hu, Bin; Liu, Rui; Li, Yanan; Nie, Ling; Zhang, Ji; Ye, Mao; Ginzburg, Yelena Z; Lin, Zhong; Yin, Biao; Chen, Huiyong; Liu, Jing.
  • Peng Y; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Liang L; Molecular Biology Research Center, Center for Medical Genetics, School of Life Sciences, Central South University, Changsha, China.
  • Zhang H; Hunan Province Key Laboratory of Basic and Applied Hematology, Central South University, Changsha, China.
  • Liu H; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Zhang G; Department of Dermatology, Xiangya Hospital, Central South University, Changsha, China.
  • Sun S; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Guo X; Department of Dermatology, Xiangya Hospital, Central South University, Changsha, China.
  • Wang Y; Department of Dermatology, Xiangya Hospital, Central South University, Changsha, China.
  • Hu B; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Liu R; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Li Y; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Nie L; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Zhang J; Molecular Biology Research Center, Center for Medical Genetics, School of Life Sciences, Central South University, Changsha, China.
  • Ye M; Department of Hematology, the Second Xiangya Hospital, Central South University, Changsha, China.
  • Ginzburg YZ; Xiangya Hospital, Central South University, Changsha, China.
  • Lin Z; Department of Rheumatology, The First Affiliated Hospital of University of South China, Hengyang, China.
  • Yin B; Molecular Science and Biomedicine Laboratory, State Key Laboratory for Chemo/Biosensing and Chemometrics, College of Biology, College of Chemistry and Chemical Engineering, Hunan University, Changsha, China.
  • Chen H; Division of Hematology and Medical Oncology, Icahn School of Medicine at Mount Sinai, New York City, New York, USA.
  • Liu J; Reproductive Hospital of Guangxi Zhuang Autonomous Region, Nanning, China.
Br J Haematol ; 201(5): 982-994, 2023 06.
Article en En | MEDLINE | ID: mdl-36872867
ABSTRACT
Beta-thalassaemia is an inherited haemoglobin disorder characterised by ineffective erythropoiesis (IE). The detailed pathogenesis of IE remains unclear. In this study, we used single-cell RNA sequencing (scRNA-seq) to examine IE in Th3/+ ß-thalassaemic mice. The results showed that the erythroid group was remarkably expanded, and genes involved in biological processes such as iron metabolism, haeme synthesis, protein folding, and response to heat were significantly upregulated from erythroid progenitors to reticulocytes in ß-thalassaemic mice. In particular, we identified a unique cell population close to reticulocytes, named ThReticulocytes, characterised by a high level of heat shock protein 70 (Hsp70) expression and dysregulation of iron metabolism and haeme synthesis signalling. Treatment of ß-thalassaemic mice with the haeme oxygenase inhibitor tin-mesoporphyrin effectively improved the iron disorder and IE, and the ThReticulocyte population and Hsp70 expression were significantly suppressed. This study revealed in detail the progression of IE at the single-cell level and possibly provided clues to find therapeutic targets in thalassaemia.
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Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Talasemia / Talasemia beta Límite: Animals Idioma: En Año: 2023 Tipo del documento: Article

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Talasemia / Talasemia beta Límite: Animals Idioma: En Año: 2023 Tipo del documento: Article