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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 645 results

SMP0390095

Missing View Pathway
Metabolite

tRNA Charging

Nevskia ramosa DSM 11499

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

  • Metabolic

    SMP0125675

    Pw127240 View Pathway
    Metabolite

    Isobutyryl-CoA Dehydrogenase Deficiency

    Homo sapiens

  • 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

    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

    SMP0120660

    Pw121916 View Pathway
    Metabolite

    2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency

    Rattus norvegicus

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

  • Disease

    SMP0120679

    Pw121935 View Pathway
    Metabolite

    beta-Ketothiolase Deficiency

    Rattus norvegicus

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

  • Disease

    SMP0120443

    Pw121694 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type III

    Mus musculus

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

  • Disease

    SMP0120503

    Pw121757 View Pathway
    Metabolite

    Isovaleric Aciduria

    Mus musculus

  • 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

    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

    SMP0125671

    Pw127236 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-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease