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Physiology

Basic Cellular

Question 157 of 180

Haemoglobin A2 (HbA2) is made up of which of the following globin chains:

Answer:

Haemoglobin A2 (HbA2), a normal variant of adult haemoglobin, makes up about 1.5 - 3.5 % of total adult haemoglobin and consists of two α and two delta (δ) globin chains.

Red blood cells contain the specialised protein haemoglobin which is responsible for carrying oxygen and carbon dioxide in the blood. Haemoglobin is composed of four polypeptide globin chains each with its own iron containing haem molecule.

Haemoglobin Synthesis

Haemoglobin synthesis occurs in immature red blood cells. Haem synthesis occurs largely in the mitochondria by a series of biochemical reactions commencing with the condensation of glycine and succinyl coenzyme A under the action of the key rate-limiting enzyme delta-aminolevulinic acid (ALA) synthase; ultimately protoporphyrin combines with iron in the ferrous (Fe2+) state to form haem. The globin chains are synthesised by ribosomes in the cytosol.

Haemoglobin Degradation

Red cells are destroyed by macrophages in the liver and spleen after ~ 120 days. The haem group is split from the haemoglobin and converted to biliverdin and then bilirubin. The iron is conserved and recycled to plasma via transferrin or stored in macrophages as ferritin and haemosiderin. An increased rate of haemoglobin breakdown results in excess bilirubin and jaundice.

Types of Haemoglobin

There are three types of haemoglobin in normal adult blood: haemoglobin A, A2 and F.

  • Normal adult haemoglobin (HbA) makes up about 96 - 98 % of total adult haemoglobin, and consists of two alpha (α) and two beta (β) globin chains.
  • Haemoglobin A2 (HbA2), a normal variant of adult haemoglobin, makes up about 1.5 - 3.5 % of total adult haemoglobin and consists of two α and two delta (δ) globin chains.
  • Foetal haemoglobin (HbF) makes up about 0.5 - 0.8 %  of total adult haemoglobin and consists of two α and two gamma (γ) globin chains.
Hb Structure Normal adult %
HbA α2β2 96 - 98%
HbA2 α2δ2 1.5 - 3.5%
HbF α2γ2 0.5-0.8%

Foetal haemoglobin is the main Hb in the later two-thirds of foetal life and in the newborn until approximately 12 weeks of age. Foetal haemoglobin has a higher affinity for oxygen than adult haemoglobin.

By OpenStax College [CC BY 3.0 (https://creativecommons.org/licenses/by/3.0)], via Wikimedia Commons

Structure of Haemoglobin. (Image by OpenStax College [CC BY 3.0 (https://creativecommons.org/licenses/by/3.0)], via Wikimedia Commons)

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  • Biochemistry
  • Blood Gases
  • Haematology
Biochemistry Normal Value
Sodium 135 – 145 mmol/l
Potassium 3.0 – 4.5 mmol/l
Urea 2.5 – 7.5 mmol/l
Glucose 3.5 – 5.0 mmol/l
Creatinine 35 – 135 μmol/l
Alanine Aminotransferase (ALT) 5 – 35 U/l
Gamma-glutamyl Transferase (GGT) < 65 U/l
Alkaline Phosphatase (ALP) 30 – 135 U/l
Aspartate Aminotransferase (AST) < 40 U/l
Total Protein 60 – 80 g/l
Albumin 35 – 50 g/l
Globulin 2.4 – 3.5 g/dl
Amylase < 70 U/l
Total Bilirubin 3 – 17 μmol/l
Calcium 2.1 – 2.5 mmol/l
Chloride 95 – 105 mmol/l
Phosphate 0.8 – 1.4 mmol/l
Haematology Normal Value
Haemoglobin 11.5 – 16.6 g/dl
White Blood Cells 4.0 – 11.0 x 109/l
Platelets 150 – 450 x 109/l
MCV 80 – 96 fl
MCHC 32 – 36 g/dl
Neutrophils 2.0 – 7.5 x 109/l
Lymphocytes 1.5 – 4.0 x 109/l
Monocytes 0.3 – 1.0 x 109/l
Eosinophils 0.1 – 0.5 x 109/l
Basophils < 0.2 x 109/l
Reticulocytes < 2%
Haematocrit 0.35 – 0.49
Red Cell Distribution Width 11 – 15%
Blood Gases Normal Value
pH 7.35 – 7.45
pO2 11 – 14 kPa
pCO2 4.5 – 6.0 kPa
Base Excess -2 – +2 mmol/l
Bicarbonate 24 – 30 mmol/l
Lactate < 2 mmol/l

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