Hemoglobin: Structure, Functions, Synthesis and Diseases

These flashcards cover key vocabulary related to the structure, functions, synthesis, and diseases associated with hemoglobin.

Hemoglobin

A tetrameric protein in red blood cells that carries oxygen from the lungs to the body tissues.

Heme

A prosthetic group of hemoglobin, consisting of a porphyrin ring and an iron atom (Fe²+).

Globin

Protein components of hemoglobin made up of polypeptide chains.

Tetrameric protein

A protein composed of four subunits, as in hemoglobin with two alpha and two beta chains.

Bohr Effect

The decrease in oxygen affinity of hemoglobin in response to a decrease in blood pH due to increased carbon dioxide concentration.

Haldane Effect

The decrease in carbon dioxide affinity of hemoglobin in response to an increase in blood pH due to increased oxygen concentration.

2,3-Bisphosphoglycerate (2,3BPG)

A molecule that regulates the binding of oxygen to hemoglobin, decreasing its affinity for oxygen.

Sickle Cell Disease

A genetic disorder caused by a mutation in the beta-globin gene, leading to abnormal hemoglobin, sickle-shaped red blood cells, and various complications.

Thalassemia

A genetic disorder characterized by reduced or absent synthesis of one of the globin chains in hemoglobin, leading to anemia.

Methemoglobinemia

A condition where hemoglobin is altered such that it cannot bind oxygen effectively, leading to tissue hypoxia.

Porphyria

A group of disorders caused by defects in the heme synthesis pathway, resulting in the accumulation of porphyrins.

Erythropoiesis

The process of producing red blood cells, primarily occurring in the bone marrow.

Iron deficiency anemia

A type of anemia characterized by a lack of iron, leading to the production of small red cells with low hemoglobin.

Fetal Hemoglobin (HbF)

The main type of hemoglobin found in the fetus, consisting of two alpha and two gamma chains, with a higher affinity for oxygen than adult hemoglobin.

Oxygen-Dissociation Curve

A graphical representation of the relationship between the partial pressure of oxygen and the saturation of hemoglobin with oxygen.

Porphyrin ring

A cyclic structure that is part of heme, formed from the linkage of four pyrrole rings.