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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 116 results

SMP0063689 View Pathway	Metabolite Valine, Leucine, and Isoleucine Degradation Mus musculus Matched Description: Valine, isoleuciine, and leucine are essential amino acids and are identified as the branched-chain … protein breakdown. In muscle, the final products of leucine, isoleucine, and valine catabolism can be … dehydrogenase (BCKD), yielding the three different CoA derivatives. Isovaleryl-CoA is produced from leucine … by these two reactions, alpha-methylbutyryl-CoA from isoleucine, and isobutyryl-CoA from valine … breakdown pathways then diverge. Leucine is ultimately converted into acetyl-CoA and acetoacetate Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0120440 View Pathway	Metabolite 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency Mus musculus Matched Description: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-Hydroxy-3-methylglutaric acidemia; Leucine Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0120723 View Pathway	Metabolite Isovaleric Aciduria Rattus norvegicus Matched Description: of leucine. A defect in this enzyme causes accumulation of ammonia, ketone bodies, Isovaleryl/2 Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000522 View Pathway	Metabolite 3-Hydroxyisobutyric Aciduria Homo sapiens Matched Description: metabolism (IEM) of valine metabolism. Only 12-13 patients have been identified with this condition. It … valine and pyrimidine catabolism. This protein catalyzes the irreversible oxidative decarboxylation Matched Compounds: L-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000138 View Pathway	Metabolite 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency Homo sapiens Matched Description: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-Hydroxy-3-methylglutaric acidemia; Leucine Matched Compounds: L-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000384 View Pathway	Metabolite Methylmalonate Semialdehyde Dehydrogenase Deficiency Homo sapiens Matched Compounds: L-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0000032 View Pathway	Metabolite Valine, Leucine, and Isoleucine Degradation Homo sapiens Matched Description: Valine, isoleuciine, and leucine are essential amino acids and are identified as the branched-chain … protein breakdown. In muscle, the final products of leucine, isoleucine, and valine catabolism can be … dehydrogenase (BCKD), yielding the three different CoA derivatives. Isovaleryl-CoA is produced from leucine … by these two reactions, alpha-methylbutyryl-CoA from isoleucine, and isobutyryl-CoA from valine … breakdown pathways then diverge. Leucine is ultimately converted into acetyl-CoA and acetoacetate Matched Compounds: L-Leucine … L-Valine Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucine … Leucine … (2s)-a-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0125673 View Pathway	Metabolite 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency Homo sapiens Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0120661 View Pathway	Metabolite 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency Rattus norvegicus Matched Description: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-Hydroxy-3-methylglutaric acidemia; Leucine Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease
SMP0120439 View Pathway	Metabolite 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency Mus musculus Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Disease