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Maple Syrup Urine disorder is caused by a mutation in BCKDHA

Maple Syrup Urine disorder is caused by a mutation in BCKDHA, DBT and BCKDHB which are the three genes that are supposed to instruct protein to work together. This mutation causes individual body to processes proteins as poison because of a lack of a particular enzymes that is required to metabolize the three branched-chain amino acids (Leucine, Isoleucine and Valine) this can lead to the brain to swell up which can cause death. The Maple syrup urine disorder can be seen in Amish children and got it name because an individual who has this disorder urine smells sweet like maple syrup. Treatment for this disorder is a no protein diet and an intravenous treatment