GlutaratesAmino Acid Metabolism, Inborn ErrorsArgininosuccinic AciduriaGlutaryl-CoA DehydrogenaseBrain Diseases, Metabolic, InbornArgininosuccinic AcidMethylmalonic AcidMetabolism, Inborn ErrorsOrotic AcidMethylmalonyl-CoA MutaseMeglutolMultiple Acyl Coenzyme A Dehydrogenase DeficiencyBrain Diseases, MetabolicAmino AcidsMalonatesAdipatesOrotate PhosphoribosyltransferaseSequence Homology, Amino AcidPurine-Pyrimidine Metabolism, Inborn ErrorsArgininosuccinate LyaseOrotidine-5'-Phosphate DecarboxylaseBarth SyndromeAzauridineHomocystinuriaPropionic AcidemiaSuccinate-Semialdehyde DehydrogenaseDicarboxylic AcidsLipid Metabolism, Inborn ErrorsElectron-Transferring FlavoproteinsHypoglycinsValeratesCarnitineAmino Acid SubstitutionMevalonate Kinase DeficiencyCloning, MolecularMutationVitamin B 12Acyl-CoA DehydrogenasesButyryl-CoA DehydrogenaseArginineKetoglutaric AcidsCitratesMolecular Sequence Data2-Aminoadipic AcidGas Chromatography-Mass SpectrometryCarbohydrate Metabolism, Inborn ErrorsCobamidesAmino Acids, EssentialCarboxy-Lyases2-Aminoadipate TransaminaseGlyceric AcidsBase SequenceOrnithine Carbamoyltransferase Deficiency DiseaseHyperammonemiaMevalonic AcidNeonatal ScreeningOxidoreductases Acting on CH-CH Group DonorsAmino Acid Transport SystemsUreaAmino Acid SequenceLysineFibroblastsPhosphotransferases (Alcohol Group Acceptor)Infant, NewbornOrnithineAcyl-CoA DehydrogenaseAmmoniaAmino Acid MotifsSuccinic AcidSequence AlignmentPentosyltransferasesCarboxylic AcidsDNA Mutational AnalysisOxo-Acid-LyasesAlkyl and Aryl TransferasesMetabolic DiseasesGlycineStereoisomerismEscherichia coliPropionatesMutation, MissenseOrnithine CarbamoyltransferaseMaple Syrup Urine DiseaseAcyl Coenzyme ABinding SitesCell LineMitochondrial DiseasesVitamin B 12 DeficiencyPoint MutationAmino Acids, AromaticAlcohol OxidoreductasesMutagenesis, Site-DirectedGenetic Complementation TestAmino Acids, SulfurLiverDNA, ComplementaryPhenotypeGenes, RecessiveSequence Homology, Nucleic AcidPeptide Fragments