Bibliographic Details
| Title: |
Erythropoiesis and Gene Expression Analysis in Erythroid Progenitor Cells Derived from Patients with Hemoglobin H/Constant Spring Disease. |
| Authors: |
Wongkhammul, Narawich, Khamphikham, Pinyaphat, Tongjai, Siripong, Tantiworawit, Adisak, Fanhchaksai, Kanda, Wongpalee, Somsakul Pop, Tubsuwan, Alisa, Maneekesorn, Supawadee, Charoenkwan, Pimlak |
| Source: |
International Journal of Molecular Sciences; Oct2024, Vol. 25 Issue 20, p11246, 11p |
| Subject Terms: |
NUCLEOTIDE sequencing, HEAT shock proteins, MOLECULAR chaperones, PROGENITOR cells, CELL analysis, ERYTHROCYTE membranes |
| Abstract: |
Hemoglobin H/Constant Spring (Hb H/CS) disease represents a form of non-deletional Hb H disease characterized by chronic hemolytic anemia that ranges from moderate to severe and may lead to transfusion-dependent thalassemia. To study the underlying mechanisms of this disease, we conducted an analysis of erythropoiesis and gene expression in erythroid progenitor cells derived from CD34+ hematopoietic stem/progenitor cells from patients with Hb H/CS disease and normal controls. Twelve patients with Hb H/CS disease and five normal controls were enrolled. Peripheral blood samples were collected to isolate CD34+ hematopoietic stem/progenitor cells for the analysis of cell proliferation and differentiation. Six samples from patients with Hb H/CS disease and three controls were subsequently studied for gene expression by next generation sequencing analysis. Erythroid progenitor cells derived from patients with Hb H/CS disease exhibited a trend towards increased rates of erythroid proliferation and decreased cell viability compared to those from controls. Moreover, erythroid progenitor cells derived from patients with Hb H/CS disease demonstrated delayed terminal differentiation. Gene expression profiling revealed elevated levels of genes encoding molecular chaperones, including the heat shock protein genes (HSPs) and the chaperonin containing TCP-1 subunit genes (CCTs) in the Hb H/CS disease group. In summary, erythroid progenitor cells derived from patients with Hb H/CS disease exhibit a trend towards heightened erythroid proliferation, diminished cell viability, and delayed terminal differentiation. Additionally, the increased expression of genes encoding molecular chaperones was observed, providing information on potential underlying pathophysiological mechanisms. [ABSTRACT FROM AUTHOR] |
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| Database: |
Complementary Index |
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