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.
