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pubmed-article:16579849pubmed:abstractTextMaple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of branched-chain keto acid dehydrogenase. MSUD has several clinical phenotypes depending on the degree of enzyme deficiency. Current treatments are not satisfactory and require new approaches to combat this disease. A major hurdle in developing new treatments has been the lack of a suitable animal model.lld:pubmed
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pubmed-article:16579849pubmed:articleTitleProduction and characterization of murine models of classic and intermediate maple syrup urine disease.lld:pubmed
pubmed-article:16579849pubmed:affiliationDepartments of Anesthesiology and Pharmacology, University of Pittsburgh, Pittsburgh, PA 15261, USA. homanicsge@anes.upmc.edulld:pubmed
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