2.6 Flashcards
Haemoglobin is a protein found in red blood cells that
Carries oxygen
Sickle-cell anaemia is an inherited condition in which affected individuals produce an
Abnormal type of haemoglobin called haemoglobin S
Sickle-cell haemoglobin has a very small difference in the
Order of amino acids in two of the polypeptide chains that make up each molecule
As a result of this difference in order of amino acids in two of the polypeptide chains, haemoglobin S is
Less soluble than normal
Crystallises in low oxygen concentrations and become sickle shaped when oxygen is low
Sickle shaped cells have difficulty in passing through small blood vessels, so they can block capillaries which means that
Less oxygenated blood reaches affected parts of the body and cells die
Causes damage to tissues and severe pain in the affected individual
Sickle cells may block the flow of blood through capillaries in the lungs, causing
- Lung tissue damage (acute chest syndrome)
- Severe pain in the arms, legs, chest and abdomen
- Strokes when parts of the brain tissue die
- Damage to most organs (kidneys and liver)
Children with sickle-cell anaemia often suffer damage to the spleen early in life, which leaves them
Vulnerable to certain bacterial infections
The sickle-shaped cells are easily damaged and are
Rapidly broken down causing anaemia
Anaemia means that the affected person has
Fewer red blood cells
Lower concentration of oxygen in their blood
People with sickle-cell anaemia are treated with:
Blood transfusions
Intravenous fluids
Antibiotics
Painkillers
However without treatment the condition is usually fatal in
Childhood
Sickle-cell anaemia is determined by a single gene with
Two alleles
Sickle cell anaemia alleles are not dominant or recessive they are
Codominant
Codominant means that
Both alleles contribute to the phenotype in a heterozygous individual
Because these Sickle cell anaemia alleles are co dominant the symbol for normal haemoglobin is
HbA