Maple syrup urine disease occurs because of a mutation in a gene subunit which causes the body to not be able to break down proteins and causes there to be retardation in the brain and body, eating disorders, vomiting, seizures and a maple syrup odor that emanates from the urine (Online Mendelian). This disease is something that is passed down through families who cannot break down the amino acids: leucine, isoleucine, and valine which further leads to them being built up in the blood (medline plus). This disorder varies in that it can be extremely severe or be very mild. The symptoms are ususally shown after birth with other forms emerfing later in a child’s years and affects nearly 1 in 185,000 children from across the world; all forms can…show more content… More specifically, the certified name of the BCKDHA gene is “branched chain keto acid dehydrogenase E1, alpha polypeptide,” with BCKDHA being the gene’s symbol, and the specific cytogenetic location that causes maple syrup urine disease is at 19q13.1-q13.2 and its molecular location is on chromosome 19: base pairs 41,397,788 to 41,425,004. The three of these genes make proteins that work as a complex by coding out instructions. This complex is what breaks down the amino acids: leucine, isoleucine, and valine. The amino acids are in protein-rich foods such as milk, meat, and eggs and a disruption in their genetic makeup can cause the protein complex in have a complete failure in its function and be able to process the foods listed above. Therefore, without a breakdown, there is an accumulation of the amino acids in the body which can be extremely harmful to the brain and other organs and can cause the deadly problems associated with maple syrup urine disease. The condition of maple syrup urine disease is recessive; thus, each cell has mutations. Even though each parent doesn’t show the symptoms or signs for having this disorder, they both carry a copy of the mutated gene and then pass it down to their offspring. The Old Order Mennonite population is more at risk for developing this disorder since there are approximately 1 in 380 newborn infants who are affected. For this specific