Hemoglobin is the protein pigment in red blood cells that acquires oxygen from the lungs to the body’s tissues and absorbs carbon dioxide from the tissues back to the lungs.
Hemoglobin is made up of 4 waxy protein globulin chains that are bounded together. The healthy adult hemoglobin (Hgb or Hb) molecule constitutes two alpha-globulin chains and a couple of beta-globulin chains. In fetuses and infants, beta-globulin chains are usually uncommon and the hemoglobin pigment is made up of two alpha chains and also two gamma chains. As the newborn grows, the gamma chains are constantly replaced by beta chains, making up the adult hemoglobin combination.
Each globulin chain composes a major iron-containing porphyrin compound called heme. Within the heme compound is an iron atom that is crucial play in carrying oxygen and carbon dioxide in our bloodstream. The iron accommodated in hemoglobin is also produce the red color in blood.
Hemoglobin also part of a crucial role by maintaining the shape of the red blood cells, i.e. biconcave shape in their natural shape. While Abnormal hemoglobin structure can, therefore, distort the shape of red blood cells and cramp their function and flow via blood vessels.
Functions of Hemoglobin:
- Hb bounds O2 transports O2 and carries the same to tissues.
- Hb binds CO2, a by product of metabolism.
- 2-3 BPG, fabricated in RBC by Rapport-Leubering shunt maintains Hb conformation at quaternary level and raises dissociation of O2 from Hb at tissue site.
- Cyanide merges with methamoglobin to make cyanomethemoglobin which is non-poisonous.
- Deep study of Hb structure provides an insight into the molecular basis of hemoglobinopathies (a genetic abnormality that causes in defect structure of one of the globin chains of the hemoglobin pigment).
Disorders of Hemoglobin:
Over the dozen of years, study of the defects of hemoglobin has provided as a paradigm for accessing insights into the molecular biology, as well as in the study of pathophysiology. To date, extent numbers of disorders of hemoglobin synthesis and/or structure have been spotted and characterized. Study of these destructive disorders has established the principle of how a abnormal genotype can change the clean function of the encoded protein, which in turn can lead to a visible clinical phenotype. Genotype/phenotype correlations have given an good understanding of pathophysiological mechanisms of disease. Let take a brief and a conceptual overview on disorders of Hemoglobin.
Anemia deficiency of red blood cells or hemoglobin:
Anemia is a haul condition that creates when your blood deficient healthy red blood cells or hemoglobin. Hemoglobin is a major portion of red blood cells and helps in biding oxygen with it. If you have too minute amount or abnormal red blood cells, or your hemoglobin is reached to abnormality level, the cells in your body will not get sufficient oxygen. Prominent symptoms or signs of anemia is, brutal fatigue occur, because organs aren’t acquiring what they require to function properly.
Sickle Cell Anemia crescent shaped genetic disorder:
Sickle cell anemia, or sickle cell disease (SCD), is a genetic disorder of the red blood cells (RBCs). Typically RBCs are sized like discs, which provides them the versatility to transport through even the tinny blood vessels. Nevertheless, with this disease, the RBCs have an irregular crescent (“sickle”) shape. That makes them waxy and firm. They can get accumulated in tiny vessels and block out blood from going distinct parts of the entire body. This can result in heavy pain and tissue damage.
SCD is an autosomal recessive sickness. You need a couple of replicates of the gene to have the disease. If you have just one copy of the gene, you are said to include sickle cell trait.
Down Syndrome defects in physical and mental traits:
Down syndrome is some physical and mental traits brought on by a gene problem that takes place before birth. Children that have Down syndrome usually have particular defects, like a flat face and a short neck. Furthermore, they have some level of intellectual disability. This differs from for everyone. But generally it is mild to moderate.
Down syndrome remains forever with you. But with proper care and nursing, children who have Down syndrome can lead to have healthy, happy and productive lives.
- Hemoglobin performs as oxygen carrier bulky.
- Responsible for red color of bloodstream.
- Carrying CO2 produce during metabolism.
- Acting as physiological active catabolites.
- Deep study over hemoglobinopathies.
- Having an interaction with drugs, i.e. Cyanide, methamoglobin etc.