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Search for 'valine AND leucine' in 'all pathways' in 'all species' returned 116 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

    SMP0125661

    Pw127226 View Pathway
    Metabolite

    Maple Syrup Urine Disease

    Homo sapiens

  • 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

    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

    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

    SMP0125658

    Pw127223 View Pathway
    Metabolite

    2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency

    Homo sapiens

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

  • Disease

    SMP0125670

    Pw127235 View Pathway
    Metabolite

    Methylmalonic Aciduria

    Homo sapiens

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

  • Disease