Which enzyme's deficiency is associated with the development of Maple Syrup Urine Disease, a condition characterized by a distinctive sweet odor of urine?

Maple Syrup Urine Disease is caused by a deficiency of the enzyme Branched-Chain Alpha-Keto Acid Dehydrogenase, which is necessary for the breakdown of certain amino acids. This deficiency leads to the accumulation of these amino acids and their metabolites in the urine, giving it a distinctive sweet odor.