Question

Explain why inborn defects of glycolytic metabolism kill MATURE red blood cells, leading to hemolytic anemia.. Note, other body cells somehow cope with these defects.

Answer 1

In Pyruvate Kinase Deficiency (one of The Inborn Error of Glycolytic Metabolism),Kill Mature Red Blood cells While other Cells somehow Cope with these Defects.

REASON:Pyruvate Kinase is the Last Enzyme Involved in Glycolytic Process,that Convert ADP into ATP & Pyruvate.Deficiency of pyruvate kinase thus results in the Blockage of Glycolytic Pathway.Hence ATP and Pyruvate are not Formed.

Since MATURE RBC lack Nucleus And Mitochondria,without a Nucleus they cannot synthesize new proteins,they are unable to produce Pyruvate Kinase Enzyme.Also,Due to the Absence of Mitochondria ,RBCs heavily depends on the anaerobic generation of ATP during Glycolysis.Due to Insufficient ATP,active process in the cells come to halt,Finally the RBC death occurs.

Since other cells have NUCLEUS and MITOCHONDRIA ,in inborn error of glycolytic metabolism,they can

1)Produce New Pyruvate Kinase Enzyme

2)Depends On mitochondria For ATP production

So they cope up with these defect.