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unreviewed

Isovaleryl-CoA dehydrogenase

1 reactions · 1 pathways

Cofactors used

ATP Bicarbonate/CO2 Biotin (B7) · B7 (Biotin) FAD · B2 (Riboflavin)

What it does, reaction by reaction

1 reactions

Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion

step 6 Irreversible/directional

Leucine-derived isovaleryl-CoA → 3-methylcrotonyl-CoA → 3-methylglutaconyl-CoA → HMG-CoA → acetoacetate + acetyl-CoA

Converts Leucine-derived isovaleryl-CoA into 3-Methylcrotonyl-CoA

› Notes

Isovaleryl-CoA dehydrogenase uses FAD, 3-methylcrotonyl-CoA carboxylase uses biotin, ATP, and bicarbonate, and downstream hydratase/lyase reactions form the final ketogenic products. Thus leucine is exclusively ketogenic.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

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Europe PMC · fetched just now · sorted by publication date

  1. 1
    SnRK1 in stress adaptation and growth regulation: Insights from <i>Arabidopsis</i> to crops.

    Zhong X, Zhang L, Han C, Bai M. · 2026-07-20

    open access unreviewed
  2. 2
    Branched-chain amino acids and gut microbiota: coregulation and impact on neurological function via the gut-brain axis.

    Li Q, Fan B, Xiao Y, Zhang Z, Zhao S, Liao B, Zeng J, Shan X, Ye H, Wen Y, Zhang L, Guo H, Xiao F, Zhu H. · 2026-07-14

    open access unreviewed
  3. 3
    Identification of novel compound heterozygous variants in isovaleric acidemia with hyperammonemia: Implications for continuous renal replacement therapy management.

    Huang H, Zhang Y, Guo L, Hu Z, Miao X, Lu C. · 2026-01-24

    open access unreviewed
  4. 4
    Rev‐erb‐α in Skeletal Muscle Adaptation: Integrating Circadian Control Mechanisms and Exercise Dynamic Responses

    Neto I, da Rocha A, Pinto A, Ramos E, Ropelle E, Pauli J, de Freitas E, da Silva A. · 2026-10-01

    open access unreviewed
  5. 5
    Mitochondria-to-plasma-membrane H<sub>2</sub>O<sub>2</sub> signaling induced by branched-chain keto acid oxidation essentially stimulates insulin secretion in pancreatic β-cells.

    Klöppel E, Engstová H, Duspiva P, Tauber J, Mozheitova O, Jabůrek M, Ježek P. · 2026-07-16

    open access unreviewed
  6. 6
    Searching for Metabolic Markers for Hypertension in Human Urine.

    Sousa A, Oliveira N, Bizkarguenaga M, de Diego A, Conde R, Embade N, Millet Ó, Verde I. · 2026-09-21

    open access unreviewed
  7. 7
    Role of the Gut-Liver-Kidney Axis in Disease Manifestation and Biomarker Alterations.

    Mashima R, Ishige N. · 2026-09-15

    open access unreviewed
  8. 8
    Integrated Transcriptomic and Metabolomic Analyses Reveal Acyl-CoA Dehydrogenase-Mediated Primordium Formation and Metabolite Synthesis in <i>Cordyceps militaris</i>.

    Zhou X, Xue L, Luo Y, Tong X, Guo J. · 2026-09-17

    open access unreviewed
  9. 9
    Paternal genetic background orchestrates fetal development through tissue-specific <i>cis</i>-regulatory divergence.

    Iqbal MA, Murani E, Hadlich F, Reyer H, Oster M, Trakooljul N, Wimmers K, Ponsuksili S. · 2026-07-13

    open access unreviewed
  10. 10
    Genes, gut microbiome and lungs: untangling the web of idiopathic pulmonary fibrosis.

    Hernandez-Beeftink T, Allen RJ, Leavy OC, Cuthbertson L, Molyneaux PL, Wain LV. · 2026-07-01

    open access unreviewed

External claims. These come from an index outside this database and are not checked against it. Treat them as leads.