Acyl-CoA dehydrogenases (BCAA branch)
Cofactors used
Clinical / pharmacological. MCAD deficiency: hypoketotic hypoglycaemia, dicarboxylic aciduria, SIDS-like presentation
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
Isoleucine-derived 2-methylbutyryl-CoA → tiglyl-CoA → 2-methyl-3-hydroxybutyryl-CoA → 2-methylacetoacetyl-CoA → acetyl-CoA + propionyl-CoA
Converts Isoleucine-derived 2-methylbutyryl-CoA into Tiglyl-CoA
› Notes
Dehydrogenase, hydratase, dehydrogenase, and thiolase reactions process the acyl chain; the first dehydrogenation uses FAD and the hydroxyacyl dehydrogenase uses NAD⁺. Propionyl-CoA is converted by propionyl-CoA carboxylase (biotin, ATP, bicarbonate), methylmalonyl-CoA epimerase, and methylmalonyl-CoA mutase (adenosylcobalamin) to succinyl-CoA, so isoleucine is both ketogenic and glucogenic.
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 · fetched just now · sorted by publication date
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1
Integrated transcriptome and metabolome reveal <i>Mycoplasma hyopneumoniae</i> -induced lipid accumulation, inflammation, oxidative stress, and energy imbalance in the liver of pigs.
Xue X, Bu Y, Yu C, Li J, Yang J, Gao M, Wang P, Gan Y, Zhang W, Fan S, Zhou R, Yin Z, Zheng X. · 2026-06-03
cited 1× open access unreviewed -
2
Hypophosphatasia: Results of a Country-Wide Selective Screening Program Using NGS Technology as a First-Tier Test.
Pushkov AA, Zhanin IS, Chudakova DA, Rusakova AA, Demianov DS, Pakhomov AV, Koroleva VB, Koshevaya YS, Burlac… · 2026-08-04
open access unreviewed -
3
GIPC1 governed ferroptosis by regulating DECR1-modulating lipid homeostasis during dilated cardiomyopathy (DCM).
Tang N, Mu R, Wang H, Wu J, Zhang J, Huang D, Han Y, Li W, Chen Y, Li X, Sun Y, Zhang Z, Zuo J, Hu Y, Yin Y, … · 2026-03-05
cited 2× open access unreviewed -
4
Placental lipid handling, growth and inflammatory pathways are modified by a maternal Mediterranean diet.
Lopez-Tello J, Youssef L, Bermejo-Poza R, Cabezas A, De la Fuente J, Crovetto F, Gratacos E, Crispi F, Sferru… · 2026-07-28
open access unreviewed -
5
CPT2 as a Convergent Node Linking Age-Associated Neuronal H3K27me3 Remodeling to Nicotinamide Mononucleotide (NMN)-Induced Expression Rescue in Metabolic Tissues.
Cheung N. · 2026-07-18
cited 1× open access unreviewed -
6
ER stress-mediated impairment of hepatic lipid export is associated with steatosis in AKI-induced remote liver injury.
Li M, Ni R, Williams K, Melaika M, Sun LE, Fu L, Subramanian V, Dhanireddy K, Liu R. · 2026-06-29
open access unreviewed -
7
Lipid Metabolic Reprogramming and Epigenetic Co-Dysregulation Across the Central Chondrosarcoma Grade Spectrum: A Multi-Cohort RNA-seq Study
Ayhan B, Dönmez N, Ayhan Z. · 2026-06-01
open access unreviewed -
8
Analysis of differentially expressed genes related to acute lung injury and their role in metabolic pathways: An integrative study using GEO data.
Yang Y. · 2026-05-01
open access unreviewed -
9
Natural Plant-Derived Compounds Targeting Oxidative Stress and Inflammation in NAFLD-Mechanisms and Repositioning Potential.
Theodorou RE, Vrettos N, Theodosis-Nobelos P. · 2026-04-29
cited 1× open access unreviewed -
10
Integrated Analysis of Transcriptome and Metabolome Reveals Molecular Responses to Ammonia Stress in the Gills of <i>Litopenaeus vannamei</i> Under Low-Salinity Conditions.
Zhao Y, Ding Y, Zhou F, Hu X, Yang Q, Cao Y. · 2026-04-13
open access unreviewed
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