Alterations of glycolysis in pyruvate kinase deficiency

This diagram of the glycolysis pathway demonstrates accumulation of metabolic intermediates in PK and hexokinase deficiencies.

In PK deficiency there is accumulation of PEP and 2,3-BPG. Elevated 2,3-BPG ameliorates the symptoms of anemia by increasing oxygen delivery to the tissues via a shift in the hemoglobin-oxygen dissociation curve.
In hexokinase deficiency 2,3-BPG does not accumulate because the enzyme acts upstream of 2,3-BPG production.

PK: pyruvate kinase; 2,3-BPG: 2,3-bisphosphoglycerate (also called 2,3-diphosphoglycerate [DPG]); PEP: phosphoenolpyruvate.

Graphic 83968 Version 6.0

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