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Bookmark or Share- Maple Syrup Urine Disease - Cleveland ClinicMaple syrup urine disease (MSUD) is a lifelong and potentially life-threatening inherited metabolic disorder. Metabolic disorders cause problems with how your body breaks down food into the tiny components it uses for energy.
- Maple Syrup Urine Disease - GeneReviews® - NCBI BookshelfMaple syrup urine disease (MSUD) is caused by decreased activity of the branched-chain alpha-ketoacid dehydrogenase complex (BCKD), the second enzymatic step in the degradative pathway of the branched-chain amino acids (BCAAs), which includes leucine, isoleucine, and valine.
- Maple syrup urine disease - WikipediaMaple syrup urine disease (MSUD) is a rare, inherited metabolic disorder that affects the body's ability to metabolize amino acids due to a deficiency in the activity of the branched-chain alpha-ketoacid dehydrogenase (BCKAD) complex. [3]
- Maple Syrup Urine Disease (MSUD) - Children's Hospital of PhiladelphiaMaple syrup urine disease is a life-threatening genetic disorder caused by the body’s inability to process amino acids and named for its distinctive odor in urine.
- Maple syrup urine disease - MedlinePlusMaple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants' urine.
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