Are Mitochondria a Potential Target for Treating β-Thalassemia? DOI Open Access
Elena Di Pierro, Valeria Di Stefano,

Margherita Migone De Amicis

и другие.

Journal of Clinical Medicine, Год журнала: 2025, Номер 14(4), С. 1095 - 1095

Опубликована: Фев. 8, 2025

The inherited genetic disorder β-thalassemia affects the hematopoietic system and is caused by low production or absence of adult hemoglobin (HbA). Ineffective erythropoiesis hallmark pathophysiology characterized an erythropoietin-driven substantial increase in erythroblast proliferation, coupled with late-stage precursor apoptosis, which results levels circulating mature red blood cells (RBCs) chronic anemia. Mitochondrial dysfunction commonly occurs these because increased demand for energy need to manage abnormal chain synthesis. Moreover, several studies have highlighted importance gradual mitochondrial clearance erythroid cell production. This review offers overview role essential cellular processes, particularly those crucial maintaining RBC health function. Additionally, recent evidence regarding contribution severity discussed, along updated insights into indirect mitochondria-targeting treatments, present potential pharmacological targets.

Язык: Английский

Are Mitochondria a Potential Target for Treating β-Thalassemia? DOI Open Access
Elena Di Pierro, Valeria Di Stefano,

Margherita Migone De Amicis

и другие.

Journal of Clinical Medicine, Год журнала: 2025, Номер 14(4), С. 1095 - 1095

Опубликована: Фев. 8, 2025

The inherited genetic disorder β-thalassemia affects the hematopoietic system and is caused by low production or absence of adult hemoglobin (HbA). Ineffective erythropoiesis hallmark pathophysiology characterized an erythropoietin-driven substantial increase in erythroblast proliferation, coupled with late-stage precursor apoptosis, which results levels circulating mature red blood cells (RBCs) chronic anemia. Mitochondrial dysfunction commonly occurs these because increased demand for energy need to manage abnormal chain synthesis. Moreover, several studies have highlighted importance gradual mitochondrial clearance erythroid cell production. This review offers overview role essential cellular processes, particularly those crucial maintaining RBC health function. Additionally, recent evidence regarding contribution severity discussed, along updated insights into indirect mitochondria-targeting treatments, present potential pharmacological targets.

Язык: Английский

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