MitochondriaAmino Acyl-tRNA SynthetasesMitochondria, LiverGlutamate-Ammonia Ligase2',5'-Oligoadenylate SynthetaseCitrate (si)-SynthaseSuccinate DehydrogenaseElectron Transport Complex IVCarbamoyl-Phosphate Synthase (Ammonia)Peptide SynthasesKineticsMolecular Sequence DataCoenzyme A LigasesMitochondria, MuscleMalate DehydrogenaseAmino Acid SequenceEscherichia coli5-Aminolevulinate SynthetaseCoenzyme A-TransferasesMitochondria, HeartLigases3-Hydroxyacyl CoA DehydrogenasesTransaminasesAspartate-tRNA LigaseMethionine-tRNA LigaseTryptophan-tRNA LigaseGlutamate DehydrogenaseLiverOrnithine-Oxo-Acid TransaminaseCarbon-Nitrogen LigasesBase SequenceMutationIsoleucine-tRNA LigaseMitochondrial ProteinsAspartate-Ammonia LigasePhenylalanine-tRNA LigaseTyrosine-tRNA LigaseDisulfiramCytosolPhosphotransferases (Carboxyl Group Acceptor)Acetyl-CoA HydrolaseIsovaleryl-CoA DehydrogenaseLeucine-tRNA LigaseSerine-tRNA LigaseValine-tRNA LigaseAldehyde DehydrogenaseLeucine TransaminasePhosphotransferasesArgininosuccinate SynthaseAldehyde OxidoreductasesAcetate-CoA LigaseDNA, MitochondrialGlutamate-tRNA LigaseTransfer RNA AminoacylationMethylmalonyl-CoA MutaseSubstrate SpecificityAdenosine TriphosphateSuccinate-CoA LigasesThreonine-tRNA LigaseAlanine-tRNA LigaseMethylmalonyl-CoA DecarboxylaseAmide SynthasesGlycine-tRNA LigaseMultienzyme ComplexesOrnithine CarbamoyltransferaseKetoglutarate Dehydrogenase ComplexCloning, MolecularRibose-Phosphate PyrophosphokinaseGlutathione SynthaseCarnitine O-AcetyltransferaseIsoenzymesAdenylosuccinate SynthaseDimethylglycine DehydrogenaseMolecular WeightBinding SitesSaccharomyces cerevisiaeOxo-Acid-LyasesThiosulfate SulfurtransferaseAcyl-CoA DehydrogenasesMuscle, SkeletalProline OxidaseHistidine-tRNA LigaseAmino AcidsAmino Acid Metabolism, Inborn ErrorsOxidation-ReductionOxygen ConsumptionGlutamineHydroxybutyrate DehydrogenaseCarbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing)Monoamine OxidaseOxidative PhosphorylationGene Expression Regulation, EnzymologicGlutamate-Cysteine LigaseOxidoreductasesArginine-tRNA LigaseAcyltransferasesSequence Homology, Amino AcidAdrenodoxinElectron Transport Complex IRNA, Messenger