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Related Concept Videos

Hematopoiesis01:21

Hematopoiesis

The process of blood cell formation is called hematopoiesis. Hematopoiesis starts early during development, on the seventh day of embryogenesis. This phase of hematopoiesis is called the primitive wave, wherein the extraembryonic yolk sac allows the production of erythroid cells and endothelial cells from a common precursor called hemangioblast. The erythroid cells provide oxygen to support the growth of the rapidly dividing embryo. Hemangioblasts later develop into hematopoietic stem cells or...
Overview of Hematopoiesis01:20

Overview of Hematopoiesis

Hematopoiesis, or blood cell production, is a vital biological process that begins early in embryonic development and continues throughout life. This process generates the various types of cells found in blood, including red blood cells, white blood cells, and platelets from hematopoietic stem cells (HSCs).
Developmental Phases of Hematopoiesis
Initially, HSCs are formed in the embryonic yolk sac, a critical site for early blood cell production. These stem cells subsequently migrate to other...
Hemoglobin01:24

Hemoglobin

Hemoglobin is a globular protein made up of four subunits. Two of these subunits are alpha chains, and the other two are beta chains. Each subunit contains a molecule of heme, which has an iron atom and can bind to oxygen. When an oxygen molecule binds to one heme group, it changes the shape of hemoglobin, making it easier for the other heme groups to bind oxygen as well.
When all four heme groups are bound to oxygen, the resulting molecule is called oxyhemoglobin. As a result, arterial blood...
The ABO Blood Group01:12

The ABO Blood Group

The ABO blood group system is a critical element of transfusion medicine, essential for determining blood compatibility in transfusions and organ transplants. It is based on specific antigens, or agglutinogens, present on the surface of red blood cells (RBCs) and corresponding antibodies, or agglutinins, in the blood plasma.
Antigens in the ABO Blood Group System
Antigens are substances that can trigger an immune response, leading to the production of antibodies. In the ABO blood group system,...
Bone Marrow Sampling and Transplants01:22

Bone Marrow Sampling and Transplants

Bone marrow transplant is a potential cure for several diseases, including cancer and specific genetic disorders. Notably, this procedure is applicable for patients suffering from aplastic anemia, certain types of leukemia, severe combined immunodeficiency disease (SCID), Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma, thalassemia, sickle-cell disease, and certain cancers.
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy the...
Oxygen Transport in the Blood01:27

Oxygen Transport in the Blood

Hemoglobin (Hb) is a crucial molecule in the human body, consisting of four polypeptide chains, each bound to an iron-containing heme group. This unique structure enables hemoglobin to bind to oxygen, with each molecule capable of combining with four molecules of oxygen, leading to rapid and reversible oxygen loading. When fully loaded with oxygen, it is called oxyhemoglobin, while hemoglobin that has released oxygen is called reduced hemoglobin or deoxyhemoglobin. As hemoglobin binds oxygen,...

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Related Experiment Video

Updated: May 9, 2026

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies
11:59

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies

Published on: September 6, 2017

Free alpha-globin pool in human bone marrow.

F M Gill, E Schwartz

    The Journal of Clinical Investigation
    |December 1, 1973
    PubMed
    Summary
    This summary is machine-generated.

    Free alpha-globin chains accumulate in the bone marrow of patients with severe beta-thalassemia and related hemoglobinopathies. This pool may play a role in regulating globin synthesis or compensating for beta-chain deficits.

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    Identifying Bone Marrow Microenvironmental Populations in Myelodysplastic Syndrome and Acute Myeloid Leukemia

    Published on: November 10, 2023

    Area of Science:

    • Hematology
    • Molecular Biology
    • Genetics

    Background:

    • Hemoglobinopathies, such as beta-thalassemia and sickle cell disease, result from genetic defects in hemoglobin synthesis.
    • Imbalances in globin chain production, particularly alpha- and beta-globin, are central to the pathophysiology of these disorders.
    • The presence and function of free globin chains in bone marrow are not fully understood.

    Purpose of the Study:

    • To investigate the presence and quantity of free alpha-globin chains in the bone marrow of individuals with various hemoglobinopathies.
    • To explore the potential relationship between free alpha-globin pools and globin chain synthesis imbalances.
    • To examine the role of free alpha-globin in potential regulatory or compensatory mechanisms.

    Main Methods:

    • Analysis of bone marrow samples from healthy controls and patients with beta-thalassemia trait, sickle beta-thalassemia, hemoglobin (Hb) Lepore trait, alpha-beta-thalassemia, homozygous beta-thalassemia, and compound heterozygotes.
    • Quantification of newly synthesized alpha-chains within the free alpha-globin pool using radioisotope labeling.
    • Assessment of beta- and alpha-globin synthesis balance in bone marrow.

    Main Results:

    • A pool of free alpha-globin chains was detected in all studied groups, including controls.
    • Patients heterozygous for thalassemia or Hb Lepore showed a significantly higher percentage (33.0%) of newly synthesized alpha-chains in the free pool compared to controls (6.2%).
    • Patients with homozygous beta-thalassemia and one compound heterozygote exhibited a marked deficit in beta-chain synthesis and a large pool of free alpha-chains.

    Conclusions:

    • Free alpha-globin chains are present in the bone marrow and their accumulation is pronounced in severe beta-thalassemia and related conditions with unbalanced globin synthesis.
    • The function of the free alpha-globin pool remains unknown but may be implicated in the regulation of globin chain synthesis.
    • This pool might also play a compensatory role in beta-chain deficient states observed in heterozygous thalassemia or Hb Lepore patients.