Explain the effect of the following on RBC survival
a. An absence of G6PD
b. An absence of PK
c. A defect in spectrin
d. A defect in ankyrin
What will be an ideal response?
Answer: (a) An absence of G6PD: RBC has a shortened life span resulting from impaired reduction of cellular oxidants via the hexose monophosphate (HMP) shunt. RBC is prone to oxidative damage that causes premature lysis.
(b) An absence of PK: RBC has a shortened life span resulting from impaired glycolysis and production of ATP via the Embden-Meyerhof (glycolytic) pathway.
(c)A defect in spectrin: Spectrin is a peripheral protein involved in RBC deformability. Defects in the spectrin molecule produce a rigid RBC that is not deformable and has a shortened life span because of an increased surface-volume ratio.
(d)A defect in ankyrin: Ankyrin is a peripheral protein responsible for RBC deformability. Defects in the ankyrin molecule produce a rigid RBC that is not deformable and has a shortened life span because of an increased surface-volume ratio.
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