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Search for 'valine AND leucine' in 'all pathways' in 'all' returned 483 results

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)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0125662

    Pw127227 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-Valine … L-Leucine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0125669

    Pw127234 View Pathway
    Metabolite

    Methylmalonate Semialdehyde Dehydrogenase Deficiency

    Homo sapiens

  • Matched Compounds: L-Valine … L-Leucine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0125674

    Pw127239 View Pathway
    Metabolite

    3-Hydroxyisobutyric Aciduria

    Homo sapiens

  • Matched Description: metabolism (IEM) of valine metabolism. Only 12-13 patients have been identified with this condition. It … valine and pyrimidine catabolism. This protein catalyzes the irreversible oxidative decarboxylation

  • Matched Compounds: L-Valine … L-Leucine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0125676

    Pw127241 View Pathway
    Metabolite

    Isovaleric Acidemia

    Homo sapiens

  • 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)-valine … Valine … L-valine … (2s)-alpha-leucine … (s)-(+)-leucine

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0125657

    Pw127222 View Pathway
    Metabolite

    beta-Ketothiolase Deficiency

    Homo sapiens

  • Matched Compounds: L-Valine … L-Leucine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0000199

    Pw000064 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-Leucine … L-Valine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0000200

    Pw000069 View Pathway
    Metabolite

    Methylmalonic Aciduria

    Homo sapiens

  • Matched Compounds: L-Leucine … L-Valine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0000523

    Pw000499 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-Leucine … L-Valine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease

    SMP0120537

    Pw121793 View Pathway
    Metabolite

    Propionic Acidemia

    Mus musculus

  • Matched Compounds: L-Valine … L-Leucine

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

    • BioPAX SVG SBGN SBML PWML All files (.zip)

    Disease