Review Question - QID 210982

A 3-month-old girl is brought to the emergency department in respiratory distress after her parents noticed that she was having difficulty breathing. They say that she developed a fever 2 days ago and subsequently developed increasing respiratory difficulty, lethargy, and productive cough. On presentation, her temperature is 103°F (39.5°C), blood pressure is 84/58 mmHg, pulse is 141/min, and respirations are 48/min. Physical exam reveals subcostal retractions and consolidation in the right lower lung field. She is also found to have coarse facial features and restricted joint movement. Serum laboratory tests reveal abnormally elevated levels of lysosomal enzymes circulating in the blood. The enzyme that is most likely defective in this patient has which of the following substrates?