Key Fact Being Tested: Pyruvate Kinase (PK) is the only enzyme in glycolysis that occurs AFTER the formation of 1,3-BPG

Normally, Glucose -->--> G3P --> 1,3-BPG --> 3-PG -->--> Pyruvate

In this case, 1,3-BPG cannot be converted to 3-PG.

Thus, 1,3-BPG is shunted to 2,3-BPG. This step bypasses ATP production. Since rbc's rely on glycolysis for ATP, this leads to hemolysis"

Pyruvate Kinase defect leads to decreased ATP leading to rigid RBCs and extra vascular hydrolysis. Increased levels of 2,3-BPG decreases hemoglobin affinity for O2.

For this one you just had to know the glycolysis pathway. Stem told you 2,3-BPG is elevated, which is upstream of pyruvate kinase.

• Normocytic Intrinsic Hemolytic Anemia: Reticulocyte >>2.5%
• PEP --> Pyruvate (last step of Glycolysis) second ATP producing step
• Mature RBC require pyruvate kinase without it they depend on the anaerobic generation of ATP
• With insufficient ATP, all active processes in the cell come to a halt. Sodium potassium ATPase pumps are the first to stop: increase intracellular K+, water leaves cell with Na+. Cell Shrinks & dies. Body is deficient in RBC + destroyed by lack of ATP = Hemolytic Anemia
• Inheritance: AR