Isovaleryl-CoA dehydrogenase
Cofactors used
What it does, reaction by reaction
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
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
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · from cache · sorted by publication date
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1
Cross-regulatory mechanisms linking ferroptosis, epigenetics, and circadian rhythm to mitochondrial quality control in diabetic cardiomyopathy.
Liu X, Ma Y, Wang X, Zhang X, Shen C, Su Y, Chang X, Zhou H, Liu JP. · 2025-09-30
cited 2× open access unreviewed -
2
Neurological Outcomes of Newborn Screening-Identified Isovaleric Acidemia: A Case Series Exploring Initial C5 Acylcarnitine Levels.
Kiss S, Lazarovski G, Evans M. · 2026-05-18
open access unreviewed -
3
[N-carbamylglutamate in the treatment of neonatal organic acidemia crisis: a report of five cases].
Zhao HY, Liang H, Li GY, Li YB, Liu XM, Yang YH. · 2026-05-01
unreviewed -
4
Mitochondrial β-carbonic anhydrase is a conserved metabolic rheostat for branched-chain amino acid catabolism and metabolic flexibility.
Sharma N, Sharkey TD, Brandizzi F. · 2026-03-15
open access unreviewed -
5
Transgenerational fitness and transcriptomic responses of Binodoxys communis to chlorantraniliprole.
Zhou T, Zhao Y, Wang L, Luo J, Cui J, Zhu X, Gao X, Wan S. · 2026-01-30
unreviewed -
6
Leucine catabolic enzyme AUH regulates BAT thermogenesis via PPARγ HMGylation and RNA-binding function in male mice.
Jiang H, Ni S, Li Z, Chen S, Li X, Zhang H, Shen WL, Jiang X, Luo P, Shu Y, Yuan F, Tong K, Xiao F, Guo F. · 2026-04-10
open access unreviewed -
7
A Rare Case of Isovaleric Acidemia With Hyperammonemia Caused by Compound Heterozygous <i>IVD</i> Variants: Clinical Features, Molecular Genetics, and Therapeutic Follow-Up.
Deng Z, Wu G, Chen S, He H, Zhang Y, Luo X, Liu S. · 2026-04-01
open access unreviewed -
8
Systematic Engineering of Proteases in Saccharopolyspora Spinosa Reveals Synergistic Enhancement of Spinosad Biosynthesis via Substrate Flux Optimization.
Jin D, Chen W, Yang D, Liu Q, Bai B, Dai Z, Liu X, Zhu Z, Rang J, Xia L. · 2026-01-31
open access unreviewed -
9
Structure and Substrate Specificity of Human Short-Chain Acyl-CoA Dehydrogenase and Insights into Pathogenicity of Disease-Associated Mutations.
Bai F, Li X, Ju K, Pan X, Jin Y, You Z, Zhang L, Liu Z, Zhang S, Luan X. · 2026-03-14
open access unreviewed -
10
Fundamental aspects of sucrose metabolism reveal a trophic link between <i>Rhodospirillum rubrum</i> and <i>Rhodobacter capsulatus</i>.
Gilson M, Bayon-Vicente G, Krings S, Toubeau L, Wattiez R, Leroy B. · 2026-02-13
open access unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.