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

SMP0000820

Pw000799 View Pathway
Metabolite

tRNA Charging

Escherichia coli

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

  • Metabolic

    SMP0121281

    Pw122589 View Pathway
    Metabolite

    tRNA Charging

    Pseudomonas aeruginosa

  • Matched Compounds: L-Leucine … L-Valine
  • Matched Proteins: Leucine-, isoleucine-, valine-, threonine-, and alanine-binding protein … Leucine--tRNA ligase … Valine--tRNA ligase
  • Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucineLeucine … (2s)-a-leucine

  • Metabolic

    SMP0000238

    Pw000091 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)-leucineLeucine … (2s)-a-leucine

  • Disease

    SMP0000173

    Pw000060 View Pathway
    Metabolite

    beta-Ketothiolase Deficiency

    Homo sapiens

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

  • Disease

    SMP0000237

    Pw000065 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)-leucineLeucine … (2s)-a-leucine

  • Disease

    SMP0120808

    Pw122069 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)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

  • Disease

    SMP0120516

    Pw121770 View Pathway
    Metabolite

    Maple Syrup Urine Disease

    Mus musculus

  • 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

    SMP0120442

    Pw121693 View Pathway
    Metabolite

    3-Methylglutaconic Aciduria Type I

    Mus musculus

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

  • Disease

    SMP0120589

    Pw121845 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)-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