Hemoglobin

Hemoglobin

A protein in red blood cells that carries oxygen throughout the body.

P50

The oxygen pressure at which 50% of hemoglobin is saturated with oxygen.

Tense State (T)

The deoxygenated form of hemoglobin.

Relaxed State (R)

The oxygenated form of hemoglobin.

Bohr Effect

Phenomenon where excess hydrogen ions stabilize hemoglobin's T-state, decreasing its affinity for oxygen.

2,3-Bisphosphoglycerate (2,3-BPG)

A molecule produced in red blood cells that reduces hemoglobin's affinity for oxygen.

Sideroblastic Anemia

A condition caused by impaired heme synthesis, leading to ineffective erythropoiesis.

Acute Intermittent Porphyria

A genetic disorder characterized by episodes of abdominal pain, neuropathy, and psychiatric symptoms due to porphyrin abnormalities.

Alpha Thalassemia

A genetic disorder resulting from mutations in alpha globin genes, leading to reduced alpha globin production.

Beta Thalassemia

A genetic disorder caused by mutations in beta globin genes, leading to excess unpaired alpha chains.

Sickle Cell Anemia

A genetic disorder resulting from a mutation in the beta-globin chain that leads to rigid, sickle-shaped red blood cells.

Hemoglobin H Disease

A type of alpha thalassemia characterized by the formation of hemoglobin H from excess beta globin chains.

Hemoglobin Bart's Disease

A severe form of alpha thalassemia caused by absent alpha globin chains, leading to the formation of gamma chain tetramers.

Hemoglobin Electrophoresis

A diagnostic test that separates different types of hemoglobin based on their electrical charge.

High-Performance Liquid Chromatography (HPLC)

A method used to separate and quantify different hemoglobin types in blood samples.

Mitochondria

Cell organelles where heme synthesis begins.

Erythropoiesis

The production of red blood cells.

Hyperbilirubinemia

An excess of bilirubin in the blood, often seen in hemolytic anemias.

Vaso-occlusive Crisis

A painful complication of sickle cell disease caused by blockage of blood vessels.

Sickle Cell Trait

A condition where an individual carries one sickle cell gene and one normal gene, typically asymptomatic.

Aplastic Crisis

A sudden decrease in red blood cell production, often triggered by infection in sickle cell disease.