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Search for 'valine AND leucine' in 'all pathways' in 'Homo sapiens' returned 68 results

SMP0125661

Pw127226 View Pathway
Disease

Maple Syrup Urine Disease

  • 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
  • SMP0125675

    Pw127240 View Pathway
    Disease

    Isobutyryl-CoA Dehydrogenase Deficiency

  • 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
  • SMP0125658

    Pw127223 View Pathway
    Disease

    2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency

  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine
  • SMP0125670

    Pw127235 View Pathway
    Disease

    Methylmalonic Aciduria

  • Matched Compounds: L-Valine … L-Leucine
  • Matched Compound Synonyms: (s)-valineValine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine
  • SMP0000237

    Pw000065 View Pathway
    Disease

    3-Methylcrotonyl-CoA Carboxylase Deficiency Type I

  • 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
  • SMP0000173

    Pw000060 View Pathway
    Disease

    beta-Ketothiolase Deficiency

  • Matched Compounds: L-Leucine … L-Valine
  • Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucineLeucine … (2s)-a-leucine
  • SMP0000238

    Pw000091 View Pathway
    Disease

    Isovaleric Aciduria

  • 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
  • SMP0000137

    Pw000061 View Pathway
    Disease

    2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency

  • Matched Compounds: L-Leucine … L-Valine
  • Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucineLeucine … (2s)-a-leucine
  • SMP0000139

    Pw000066 View Pathway
    Disease

    3-Methylglutaconic Aciduria Type I

  • Matched Compounds: L-Leucine … L-Valine
  • Matched Compound Synonyms: (2s)-alpha-leucine … (s)-(+)-leucine … (s)-leucineLeucine … (2s)-a-leucine
  • SMP0125664

    Pw127229 View Pathway
    Disease

    3-Methylglutaconic Aciduria Type I

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