Review Question - QID 109661

A 23-year-old woman is seen by her primary care physician for fatigue. She says that she has always felt a little short of breath compared to her friends; however, she did not think that it was abnormal until she started trying a new exercise regimen. On physical exam, she is found to have mild conjunctival pallor and a peripheral blood smear is obtained showing echinocytes but no intracellular accumulations. Upon further questioning, she recalls that several relatives have had similar issues with fatigue and pallor in the past. Which of the following is true about the rate limiting enzyme of the biochemical pathway that is affected by this patient's most likely condition?