Maple Syrup Urine Disease is a genetic disorder which is characteried with in 12 to 24 hours of birth by a sweet scented earwax. It gets its name because it causes an infants urine to have a distinct smell of something sweet such as burned caramel. Occuring in only 1 in every 185,000 infants worldwide, MSUD is very rare. Although the title for MSUD gives it a some what humerous apperance, this disease is actually deadly. It seriouly affects how three amino Acids are broken down. these three are leucine, isoleucine, and valine. Besides for being used to make protiens these three amino acids are also very important when it comes to energy. When these Amino Acids fail to be broken down by the BCKDHA, BCKDHB, DBT, and DLD genes, they begin to build up. These build ups begin to cause harm to the body due to the fact that they are toxic to the brain and other organs.