Maple Syrup Urine DiseaseKidney Tubules3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)Ketone OxidoreductasesKidney Tubules, ProximalKidneyKidney Tubules, CollectingAmino Acids, Branched-ChainKeto AcidsNorleucineUrineMultienzyme ComplexesIsoleucineKidney CortexLeucineNephronsPhenylketonuriasKidney Tubules, DistalKidney MedullaThiamineGlutamineMalpighian TubulesSodium-Potassium-Exchanging ATPaseEpitheliumKidney DiseasesMolecular Sequence DataNutritional RequirementsGluconeogenesisMutationKidney TransplantationTissue DistributionMicroscopy, ElectronEpithelial CellsDietary ProteinsDogsImmunohistochemistryBase Sequence