All are characteristics of Maple Syrup Urine Disease EXCEPT:
A) hereditary enzyme defect.
B) elevated levels of val, leu, and ile and their corresponding α-keto acids in blood and urine.
C) restrictive intake of proteins with val, leu and ile is required.
D) fatal without detection and treatment.
E) elevated blood levels of acetoacetate and β-hydroxybutyrate.
Correct Answer:
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