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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 483 results

SMP0000237 View Pathway	Metabolite 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I Homo sapiens 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-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
SMP0000238 View Pathway	Metabolite Isovaleric Aciduria Homo sapiens Matched Description: of leucine. A defect in this enzyme causes accumulation of ammonia, ketone bodies, Isovaleryl/2 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
SMP0120589 View Pathway	Metabolite Isovaleric Acidemia Mus musculus Matched Description: (IEM) of leucine metabolism. It is an autosomal recessive disorder that is caused by a deficiency of … the conjugate. A diet that is also restricted in leucine consumption is also useful in treating the disorder. 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
SMP0120808 View Pathway	Metabolite Isovaleric Acidemia Rattus norvegicus Matched Description: (IEM) of leucine metabolism. It is an autosomal recessive disorder that is caused by a deficiency of … the conjugate. A diet that is also restricted in leucine consumption is also useful in treating the disorder. 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
SMP0125488 View Pathway	Metabolite Immunometabolism Pathway (Viral Activation) Homo sapiens Matched Description: reduction in essential amino acids (threonine, lysine, tryptophan, leucine, isoleucine, valine 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
SMP0125493 View Pathway	Metabolite Bacterial Sepsis Homo sapiens Matched Description: , leucine, isoleucine, valine, arginine) and a mild reduction in gluconeogenic acids (glycine, serine 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
SMP0468325 View Pathway	Metabolite Secondary Metabolites: Valine and L-Leucine Biosynthesis from Pyruvate Raoultella planticola ATCC 33531 Matched Description: The biosynthesis of Valine and L-leucine from pyruvic acid starts with pyruvic acid interacting … -oxovaleric acid can produce an L-valine by interacting with a L-glutamic acid through a Valine … Transaminase resulting in the release of a Oxoglutaric acid and a L-valine. The 3-methyl-2-oxovaleric acid … with a L-glutamic acid through a branched-chain amino-acid aminotransferase resulting in the oxoglutaric acid and L-leucine. Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … Keto-leucine … (2s)-alpha-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0468637 View Pathway	Metabolite Secondary Metabolites: Valine and L-Leucine Biosynthesis from Pyruvate Acetomicrobium hydrogeniformans Matched Description: The biosynthesis of Valine and L-leucine from pyruvic acid starts with pyruvic acid interacting … -oxovaleric acid can produce an L-valine by interacting with a L-glutamic acid through a Valine … Transaminase resulting in the release of a Oxoglutaric acid and a L-valine. The 3-methyl-2-oxovaleric acid … with a L-glutamic acid through a branched-chain amino-acid aminotransferase resulting in the oxoglutaric acid and L-leucine. Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … Keto-leucine … (2s)-alpha-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0464720 View Pathway	Metabolite Secondary Metabolites: Valine and L-Leucine Biosynthesis from Pyruvate Escherichia coli HS Matched Description: The biosynthesis of Valine and L-leucine from pyruvic acid starts with pyruvic acid interacting … -oxovaleric acid can produce an L-valine by interacting with a L-glutamic acid through a Valine … Transaminase resulting in the release of a Oxoglutaric acid and a L-valine. The 3-methyl-2-oxovaleric acid … with a L-glutamic acid through a branched-chain amino-acid aminotransferase resulting in the oxoglutaric acid and L-leucine. Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … Keto-leucine … (2s)-alpha-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic
SMP0464742 View Pathway	Metabolite Secondary Metabolites: Valine and L-Leucine Biosynthesis from Pyruvate Escherichia coli str. K-12 substr. DH10B Matched Description: The biosynthesis of Valine and L-leucine from pyruvic acid starts with pyruvic acid interacting … -oxovaleric acid can produce an L-valine by interacting with a L-glutamic acid through a Valine … Transaminase resulting in the release of a Oxoglutaric acid and a L-valine. The 3-methyl-2-oxovaleric acid … with a L-glutamic acid through a branched-chain amino-acid aminotransferase resulting in the oxoglutaric acid and L-leucine. Matched Compounds: L-Valine … L-Leucine Matched Compound Synonyms: (s)-valine … Valine … L-valine … Keto-leucine … (2s)-alpha-leucine BioPAX SVG SBGN SBML PWML All files (.zip) Metabolic