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unreviewed

HMG-CoA lyase (HMGCL)

1 reactions · 1 pathways

Clinical / pharmacological. HMG-CoA lyase deficiency: hypoketotic hypoglycaemia with metabolic acidosis

What it does, reaction by reaction

1 reactions

Ketogenesis Lipid Metabolism · Liver mitochondrial matrix

step 3 Irreversible/directional

HMG-CoA → acetoacetate + acetyl-CoA

Converts HMG-CoA into Acetoacetate Acetyl-CoA

› Notes

HMG-CoA lyase cleaves HMG-CoA to the first ketone body, acetoacetate, and acetyl-CoA. No nucleotide cofactor is required.

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
    Clinical trajectory and long-term management of 3-Hydroxy-3-Methylglutaryl-CoA lyase deficiency: Insights from a lifespan cohort.

    Calisgan K, Uygur E, Bayhan B, Aghakishili HB, Soylu EI, Col TA, Akbulut S, Yesil SA, Cansever MŞ, Zubarioglu… · 2026-08-05

    unreviewed
  2. 2
    Impaired Lung BCAA Metabolism Promotes Ferroptosis and Resultant Pulmonary Arterial Hypertension-Associated Hepatopathy.

    Blake MJ, Hong J, Brownstein A, Rhodes CJ, Swietlik EM, Blake JC, Moon RA, Hartweck LM, Mendelson JB, Prisco … · 2026-05-30

    open access unreviewed
  3. 3
    β-Hydroxybutyrate Inhibits Angiogenesis, Suppresses Non-Small Cell Lung Cancer Growth, and Enhances Gemcitabine Antitumor Activity.

    Labanie Y, Arafat K, Sulaiman S, Al-Azawi AM, Attoub S. · 2026-06-04

    open access unreviewed
  4. 4
    Hepatoprotective Potential of Walnut Oil Unsaponifiable Matter on Aging-Induced Liver Injury via Gut Microbiota-Liver Axis.

    Zhou J, Hong D, Bai R, Wang X, Zhu S, Tian Q, Xia J, Chen Z, Li S, Jia Y, Ma A. · 2026-06-05

    open access unreviewed
  5. 5
    Hepatic ketogenesis is not required for exercise training to mitigate diet-induced liver steatosis in male mice.

    Vang CM, Ortega AF, Pfeiffer RE, Hartmann JL, Hampton GS, Wang H, Queathem ED, Crawford PA, Han X, Hughey CC. · 2026-05-14

    open access unreviewed
  6. 6
    Ketone body metabolism activates the immune response against <i>Staphylococcus aureus</i> infection by fueling the tricarboxylic acid cycle and affecting histone β-hydroxybutyrylation.

    Cai H, Yue X, Chen Y, Chen X, Qiao R, Zhong G, Rong S, Zhang L, Li Z, Liao A, Xiong W, Guo C, Zhu Y, Deng K, … · 2026-04-03

    open access unreviewed
  7. 7
    Crystal structure of 3-hydroxypropionyl-CoA synthetase (ADP-forming) from <i>Nitrosopumilus maritimus</i>.

    Johnson J, Tosun B, Yilmaz M, Tolar BB, Yoshikuni Y, Francis CA, Doukov T, Yokoi S, Wakatsuki S, DeMirci H. · 2026-03-17

    open access unreviewed
  8. 8
    Proteomic Profile of Skeletal Muscles and Liver in a Dexamethasone-Induced Atrophy Model: Insights into the Role of β-Hydroxy-β-Methylbutyrate on Contractile and Metabolic Protein.

    Ventura TMO, Tokuhara CK, Pereira M, de Souza Bueno CR, Favaretto-Junior IA, German IJS, de Oliveira Rosso MP… · 2026-01-22

    open access unreviewed
  9. 9
    Functional Precision Oncology in Fibrolamellar Carcinoma: Ex Vivo Identification of Therapeutic Vulnerabilities.

    Schneider SA, D'Amora P, Evans SS, Kent P, Stockwell T, Bakaya VS, Bernard PJ, Francisco FR, Torres L, Henry … · 2026-05-27

    open access unreviewed
  10. 10
    Knockout of filamin A in KGN granulosa tumor cells impairs proliferation, cell cycle progression, migration, and cytoskeletal organization under mechanical stress.

    Jiang Y, Caban KM, Peitzsch M, Herrmann C, Mayr D, Stöckl JB, Fröhlich T, Mayerhofer A, Müller-Taubenberger A… · 2026-05-20

    open access unreviewed

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