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process certain protein building blocks (amino acids) properly.. Maple syrup urine disease, Maple syrup urine disease (MSUD) is a metabolism disorder passed down through families in which the body cannot break down. Pediatrics 2002 Jun; 109(6): 999-1008. Nord, et al. profile of Patients with Maple Syrup Urine Disease." 1991. Maple syrup urine disease is an inherited error in the metabolism of leucine, isoleucine and valine (essential amino acids). results Image Decarboxylation of leucine. span class=fFile Format:span PDFAdobe Acrobat - a as HTMLa Continuous venovenous
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