PANCE Practice Test

Category - PA

In Tay-Sachs disease, deficiency of which enzyme is responsible for the neurologic deficits?
  1. Hexosaminidase A
  2. Glucocerebrosidase
  3. Phosphofructokinase
  4. Glucose phosphorylase
Explanation
Answer: A - Tay-Sachs disease is caused by an enzyme defect. An important enzyme, hexosaminidase A, is not completely formed. Children with Tay-Sachs disease die prematurely and exhibit mental retardation, seizures, and blindness.
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