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Review Question - QID 108548

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QID 108548 (Type "108548" in App Search)
An 8-year old boy is brought into clinic for evaluation of possible scoliosis that was newly found on a routine exam at school. On exam, he is also noted to be in the 99th percentile for height and 70th percentile for weight. He appears to have abnormally long extremities as well as an upward lens dislocation on ophthalmologic exam. A mutation leading to a defect in which of the following proteins is the most likely cause of his condition?