**Core Concept**
Decarboxylation is a crucial biochemical reaction where a carboxyl group is removed from a molecule. In the context of amino acid metabolism, this process involves the conversion of amino acids to their corresponding biogenic amines. Valine, leucine, and isoleucine are branched-chain amino acids (BCAAs) that require decarboxylation for their metabolic breakdown. This process is catalyzed by the enzyme branched-chain alpha-keto acid dehydrogenase complex (BCKDC).
**Why the Correct Answer is Right**
The correct answer is related to Maple Syrup Urine Disease (MSUD), a genetic disorder caused by a deficiency of the BCKDC enzyme. This enzyme complex is responsible for the decarboxylation of valine, leucine, and isoleucine, resulting in the accumulation of toxic intermediates. The defect in BCKDC leads to the inability to break down these BCAAs, causing their levels to rise in the blood and urine. The name "Maple Syrup Urine Disease" is due to the characteristic sweet odor of the urine in affected individuals.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because it does not directly relate to the decarboxylation defect of BCAAs.
**Option B:** This option is incorrect because it is a different metabolic disorder altogether, unrelated to BCAA decarboxylation.
**Option C:** This option is incorrect because it is a different genetic disorder that affects a different aspect of amino acid metabolism.
**Clinical Pearl / High-Yield Fact**
Maple Syrup Urine Disease is a classic example of a metabolic disorder that can be diagnosed through newborn screening programs. Early detection and treatment can prevent long-term complications and improve outcomes for affected individuals.
**Correct Answer: D. Maple Syrup Urine Disease**
Free Medical MCQs Β· NEET PG Β· USMLE Β· AIIMS
Access thousands of free MCQs, ebooks and daily exams.
By signing in you agree to our Privacy Policy.