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deficiency
anaemia and
polycythaemia
RBC metabolism
● RBCs are complex metabolically active cells using to make ATP and reducing
equivalents to ensure flexibility and O2 delivery.
● Lifespan of RBC is 120 days.
● Neither use Oxygen for Extraction of energy nor synthesizes protein
● Mainly anaerobic, RBC have to deliver not consume O2
● No nucleus /no mitochondria
● Divided into anaerobic glycolysis and Three ancillary pathways
● RBCs contain no mitochondria, so there is no respiratory chain, no citric acid
cycle, no oxidation of fatty acids or ketone bodies.
● The RBC is highly dependent upon glucose is its energy source.
● Energy in form of ATP is Obtained only from the glycolytic breakdown of glucose
with the production of Lactic acid (anaerobic glycolysis).
Glucose transport to RBC Membrane
Glucose is transported through RBC membrane by facilitated diffusion through
glucose transporters (GLUT1).
Glycolysis
Importance of Glycolysis in red cells
● Energy production : it’s the only pathway that supplies the red cell with ATP.
● Reduction of meeting methemoglobin : Glycolyses provides NADH for
reduction of methemoglobin by NADH Cytob5 reductase
● In red cells 2,3 bisphosphoglycerate binds to Hb, decreasing its affinity for
oxygen and helps its availability to tissues.
Utilisation of ATP
● Phosphorylation of sugars in proteins
● ATPase driven ion pumps
● Maintenance of membrane asymmetry
● Maintenance of red cell shape and deformability using ATP dependent
cytoskeleton
Meth haemoglobin reductase
pathway
● Maintains iron in Reduced rate for effective transport of oxygen.
● Protect SH group of haemoglobin and membrane proteins from oxidation.
Leubering rapport shunt
● Hydrogen ion concentration is the most important physiological modulater.
Leubering rapoport shunt
● Binding of 2,3DPG to deoxyhaemoglobin stabilise the tense state of
haemoglobin and favours release of oxygen
● Free 2,3 DPG also binds with band 3 and causes partial detachment of
membrane from cytoskeleton allowing lateral movement of membrane
structure.
Pentose phosphate pathway
● Production of NADPH which is reducing power
● Glutathione is needed in reduced from for :
○ Elimination of peroxidase
○ Protection of proteins SH group
● This shunt also provide ribose 5 phosphate needed for PRPP (substrate for
adenine nucleotides required for continuing ATP synthesis)

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G6pd deficiency anemia dpolycythemia.pptx

  • 2. RBC metabolism ● RBCs are complex metabolically active cells using to make ATP and reducing equivalents to ensure flexibility and O2 delivery. ● Lifespan of RBC is 120 days. ● Neither use Oxygen for Extraction of energy nor synthesizes protein ● Mainly anaerobic, RBC have to deliver not consume O2 ● No nucleus /no mitochondria ● Divided into anaerobic glycolysis and Three ancillary pathways
  • 3. ● RBCs contain no mitochondria, so there is no respiratory chain, no citric acid cycle, no oxidation of fatty acids or ketone bodies. ● The RBC is highly dependent upon glucose is its energy source. ● Energy in form of ATP is Obtained only from the glycolytic breakdown of glucose with the production of Lactic acid (anaerobic glycolysis).
  • 4. Glucose transport to RBC Membrane Glucose is transported through RBC membrane by facilitated diffusion through glucose transporters (GLUT1).
  • 5. Glycolysis Importance of Glycolysis in red cells ● Energy production : it’s the only pathway that supplies the red cell with ATP. ● Reduction of meeting methemoglobin : Glycolyses provides NADH for reduction of methemoglobin by NADH Cytob5 reductase ● In red cells 2,3 bisphosphoglycerate binds to Hb, decreasing its affinity for oxygen and helps its availability to tissues.
  • 6.
  • 7. Utilisation of ATP ● Phosphorylation of sugars in proteins ● ATPase driven ion pumps ● Maintenance of membrane asymmetry ● Maintenance of red cell shape and deformability using ATP dependent cytoskeleton
  • 8. Meth haemoglobin reductase pathway ● Maintains iron in Reduced rate for effective transport of oxygen. ● Protect SH group of haemoglobin and membrane proteins from oxidation.
  • 9. Leubering rapport shunt ● Hydrogen ion concentration is the most important physiological modulater.
  • 10. Leubering rapoport shunt ● Binding of 2,3DPG to deoxyhaemoglobin stabilise the tense state of haemoglobin and favours release of oxygen ● Free 2,3 DPG also binds with band 3 and causes partial detachment of membrane from cytoskeleton allowing lateral movement of membrane structure.
  • 11. Pentose phosphate pathway ● Production of NADPH which is reducing power ● Glutathione is needed in reduced from for : ○ Elimination of peroxidase ○ Protection of proteins SH group ● This shunt also provide ribose 5 phosphate needed for PRPP (substrate for adenine nucleotides required for continuing ATP synthesis)