Maple Syrup Urine Disease is characterized by elevated levels of which amino acids?

Maple Syrup Urine Disease (MSUD) is a metabolic disorder caused by a deficiency in the branched-chain alpha-keto acid dehydrogenase complex, leading to the accumulation of certain branched-chain amino acids in the body. The primary amino acids involved in this condition are isoleucine, leucine, and valine.

In the context of this disease, the metabolic pathway that breaks down these branched-chain amino acids is disrupted, causing their elevated levels to accumulate in the blood and subsequently being excreted in the urine, which gives the disease its characteristic scent reminiscent of maple syrup.

The other groups of amino acids listed in the options do not relate to the metabolic defect seen in Maple Syrup Urine Disease. Glycine, serine, and proline are not associated with this condition, nor are the amino acids alanine, glutamate, aspartate, lysine, histidine, or phenylalanine. Understanding the specific defect and its consequences is crucial in recognizing the clinical presentation and biochemical markers of the disease.