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

SMP0120587

Pw121843 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)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0120662

    Pw121918 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)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0120664

    Pw121920 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type III

    Rattus norvegicus

  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0120723

    Pw121982 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)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0125673

    Pw127238 View Pathway
    Metabolite

    3-Hydroxyisobutyric Acid Dehydrogenase Deficiency

    Homo sapiens

  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0063689

    Pw064671 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)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Metabolic

    SMP0000137

    Pw000061 View Pathway
    Metabolite

    2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency

    Homo sapiens

  • Matched Compounds: L-Leucine … L-Valine
  • Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucineLeucine … (2s)-a-leucine

  • Disease

    SMP0000139

    Pw000066 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type I

    Homo sapiens

  • Matched Compounds: L-Leucine … L-Valine
  • Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucineLeucine … (2s)-a-leucine

  • Disease

    SMP0125659

    Pw127224 View Pathway
    Metabolite

    Propionic Acidemia

    Homo sapiens

  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0125664

    Pw127229 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type I

    Homo sapiens

  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease