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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 96 results

SMP0000200

Pw000069 View Pathway
Metabolite

Methylmalonic Aciduria

Homo sapiens

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

  • Disease

    SMP0120523

    Pw121779 View Pathway
    Metabolite

    Methylmalonate Semialdehyde Dehydrogenase Deficiency

    Mus musculus

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

  • Disease

    SMP0120525

    Pw121781 View Pathway
    Metabolite

    Methylmalonic Aciduria

    Mus musculus

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

  • Disease

    SMP0120537

    Pw121793 View Pathway
    Metabolite

    Propionic Acidemia

    Mus musculus

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

  • Disease

    SMP0120665

    Pw121921 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type IV

    Rattus norvegicus

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

  • Disease

    SMP0120663

    Pw121919 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type I

    Rattus norvegicus

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

  • Disease

    SMP0120736

    Pw121995 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)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0120757

    Pw122018 View Pathway
    Metabolite

    Propionic Acidemia

    Rattus norvegicus

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

  • Disease

    SMP0120745

    Pw122006 View Pathway
    Metabolite

    Methylmalonic Aciduria

    Rattus norvegicus

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

  • Disease

    SMP0120807

    Pw122068 View Pathway
    Metabolite

    Isobutyryl-CoA Dehydrogenase Deficiency

    Rattus norvegicus

  • Matched Description: error of metabolism (IEM) of valine metabolism. It is an autosomal recessive disorder that is caused … the metabolism of branched-chain amino acids, specifically valine. This enzyme is responsible for … the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. Defects … in the IBD enzyme function lead to elevated levels of valine in blood and other biofluids … energy. Treatment may include the use of L-carnitine supplements, frequent meals, and a low-valine diet.
  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease