Hematopoietic stem cells (HSCs) have the ability to self-renew, differentiate into various blood cell types, and reside in the bone marrow (BM) niche. Estrogen-related receptor α (ERRα) is an orphan nuclear receptor that has a role in mitochondrial biogenesis and metabolic regulation. Previous research has shown that ERRα contributes to the development of acute myeloid leukemia (AML) by acting as a key regulator of mitochondrial processes, though its role in HSC regulation remains mostly unknown. Flow cytometric analysis determined the population of the hematopoietic stem and progenitor cells (HSPC) using Errα knockout (KO) and conditional KO (cKO) mice. Furthermore, we investigated reconstitution with BM transplantation assay. Here, the ablation of Errα in the BM demonstrated that the production of mature blood cells, lineage distribution within hematopoietic organs, and frequencies of the HSPC populations were similar to those of controls. In addition, the ablation of Errα did not perturb HSC function under the stress of transplantation. Collectively, ERRα is not necessary for the control of HSPC populations, as well as for the maintenance of HSC characteristics and functions within the BM.
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http://dx.doi.org/10.1016/j.bbrc.2024.151075 | DOI Listing |
Stem Cell Res Ther
December 2024
Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, No.107, West Yan Jiang Road, Guangzhou, 510120, Guangdong, China.
Background: Allo-HSCT is a curative therapy for patients with transfusion-dependent thalassemia (TDT). The high incidence of transplant-related complications is becoming an obstacle to safe and effective unrelated donor (URD) transplantation.
Methods: In this retrospective study, we reported the survival outcomes and complications of transplantation in thalassemia patients using a novel regimen consisting of pre-transplantation immunosuppression (PTIS) and modified myeloablative conditioning based on intravenous busulfan, cyclophosphamide, fludarabine, and rabbit anti-human thymocyte immunoglobulin.
Ocul Surf
December 2024
Department of Prof. Cochereau, A. De Rothschild Foundation Hospital, Paris, France. Electronic address:
Purpose: Graft-versus-host disease (GVHD) is a common complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). GVHD may affect several organs, including ocular manifestations, ranging from dry eye syndrome to sight-threatening corneal ulceration or perforation. Limited information is available about characteristics and treatments of ocular GVHD and its relation to general prognosis.
View Article and Find Full Text PDFCell Stem Cell
December 2024
Children's Research Institute and the Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA; Howard Hughes Medical Institute, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA. Electronic address:
Fatty acid oxidation is of uncertain importance in most stem cells. We show by C-palmitate tracing and metabolomic analysis that hematopoietic stem/progenitor cells (HSPCs) engage in long-chain fatty acid oxidation that depends upon carnitine palmitoyltransferase 1a (CPT1a) and hydroxyacyl-CoA dehydrogenase (HADHA) enzymes. CPT1a or HADHA deficiency had little or no effect on HSPCs or hematopoiesis in young adult mice.
View Article and Find Full Text PDFTransfus Apher Sci
December 2024
University of Health Sciences, Ankara Oncology Training and Research Hospital, Department of Hematology & Apheresis Unit, Ankara, Turkey; Ankara Yildirim Beyazit University, School of Medicine, Department of Internal Medicine, Division of Hematology, Ankara, Turkey.
Objectives: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative treatment for hematological diseases, with success rates improving due to advancements in conditioning regimens and new anti-graft versus host disease (GVHD) drugs. Ruxolitinib, an oral selective Janus kinase (JAK) 1 and 2 inhibitor has been used to mitigate the effects of various inflammatory and myeloproliferative syndromes, given the JAK kinase pathway's central role in cytokine signaling during inflammatory and immune processes. In this study we aimed to assess ruxolitinib's efficacy in patients with chronic GVHD (cGVHD).
View Article and Find Full Text PDFCytotherapy
December 2024
Pediatric Hematology Oncology, Hospital Vall´de Hebrón, Barcelona, Spain.
Background: The management of relapsed acute lymphoblastic leukemia (ALL) after hematopoietic stem cell transplantation (HSCT) has evolved significantly. Initially, treatment options were limited to palliative care, salvage chemotherapy, and second HSCT. Currently, the focus has shifted to innovative immunotherapies, particularly CAR T-cell therapy.
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