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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 488 results

SMP0120587 View Pathway	Metabolite 3-Hydroxyisobutyric Aciduria Mus musculus 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-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
SMP0120662 View Pathway	Metabolite 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I Rattus norvegicus Matched Description: constitute the breakdown of leucine. Mutations in the aforementioned genes leads to a reduction in the … breakdown leucine properly. Consequently, this leads to the build up of toxic byproducts which are not … processed as the breakdown of leucine is left incomplete. If these toxic byproducts manifest themselves 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
SMP0120664 View Pathway	Metabolite 3-Methylglutaconic Aciduria Type III Rattus norvegicus 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
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
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
SMP0120523 View Pathway	Metabolite Methylmalonate Semialdehyde 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
SMP0120525 View Pathway	Metabolite Methylmalonic Aciduria 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
SMP0120736 View Pathway	Metabolite Maple Syrup Urine Disease Rattus norvegicus Matched Description: for a protein which is vital in the breakdown of amino acids, specifically the amino acids leucine … , isoleucine and valine. This disorder is characterized by a large accumulation of these amino acids 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
SMP0120441 View Pathway	Metabolite 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I Mus musculus Matched Description: constitute the breakdown of leucine. Mutations in the aforementioned genes leads to a reduction in the … breakdown leucine properly. Consequently, this leads to the build up of toxic byproducts which are not … processed as the breakdown of leucine is left incomplete. If these toxic byproducts manifest themselves 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