Which aminoaciduria is associated with the overflow of branched chain amino acids?

The condition associated with the overflow of branched-chain amino acids is Maple Syrup Urine Disease (MSUD). This metabolic disorder is caused by a deficiency in a complex of enzymes responsible for the breakdown of branched-chain amino acids: leucine, isoleucine, and valine. When these amino acids are not metabolized properly, they accumulate in the blood and spill over into the urine, leading to a characteristic sweet odor that resembles maple syrup.

In Maple Syrup Urine Disease, the accumulation of these amino acids can lead to various neurological issues and metabolic derangements, particularly if not detected and treated early. The elevation of branched-chain amino acids serves as a pivotal diagnostic marker for this condition. That's why the presence of these amino acids in the urine forms the basis of the condition's name and distinguishes it from other metabolic disorders, which may not specifically involve the overflow of branched-chain amino acids.