Full 2L QBank
Biochemistry General 71504455

All of the following are excreted in urine in Maple syrup urine disease, EXCEPT:

A
Leucine
B
Isoleucine
C
Valine
D
None of the above
High-Yield Explanation
As the name implies, the odor of urine in maple syrup urine disease (branched-chain ketonuria) suggests maple syrup or burnt sugar. The biochemical defect involves the -keto acid decarboxylase complex. Plasma and urinary levels of leucine, isoleucine, valine, -keto acids, and -hydroxy acids (reduced -keto acids) are elevated. Ref: Harper 28th edition, chapter 29.

Related Biochemistry MCQs

Practice 2,00,000+ NEET PG Questions Free

Timed mock tests, mistake queue analytics, audio lectures & zero attempt limits on i❤️Exams.

Start Free Mock Test Now