HMG-CoA lyase (HMGCL)
Clinical / pharmacological. HMG-CoA lyase deficiency: hypoketotic hypoglycaemia with metabolic acidosis
What it does, reaction by reaction
Ketogenesis Lipid Metabolism · Liver mitochondrial matrix
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
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
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 -
2
Research progress in metabolic reprogramming and targeting metabolic pathways for clear cell renal cell carcinoma.
Yao JT, Hu PC, Li XW, Xu JC, Wang KJ, Ye SZ, Meng XY, Chen HC, Liang Y, Ma Q. · 2025-12-19
open access unreviewed -
3
Human brown fat metabolism associates with systemic branched-chain amino acids homeostasis.
U-Din M, Acosta FM, Saari T, de Mello Laaksonen VD, van der Kolk BW, Monfort-Pires M, Clemente-Postigo M, Nie… · 2026-08-26
open access unreviewed -
4
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 -
5
Parvovirus B19 and Cellular Transcriptome Dynamics in UT7/EpoS1 Cells.
Guglietta N, Bichicchi F, Gasperini I, Manaresi E, Gallinella G. · 2026-09-08
open access unreviewed -
6
Propionic Acidemia: An Ambispective Cohort Study on Phenotypic and Genotypic Characteristics at a Tertiary Center in Vietnam.
Quang DM, Vu CD, Nguyen KN, Nguyen NL, Mai TTC, Huong NTT, Mo DT, Phuong LT, Tran TH, Tu NH, Khoa NX, Vuong V… · 2026-09-11
open access unreviewed -
7
Multi-Omics Mendelian Randomization Maps Lipid-Related Brain Cell-Type Associations with Kidney Disease.
Deng J, Cao B, Zheng F, Ai S, Wang Y, Wang X. · 2026-09-11
open access unreviewed -
8
GSK3β and Plk1 sequentially phosphorylate ATP-citrate lyase to promote homologous recombination.
Zhou S, Zhou X, Chen Q, Yuan X, Zhu S, Huang J, Wang F, Yan H. · 2026-09-09
open access unreviewed -
9
Histone Post-Translational Modifications Regulating Ferroptosis: The Molecular Mechanisms and Disease Associations.
Tang X, Luo H, Gao S, Liu X, Sun Q, Zeng Y. · 2026-09-01
open access unreviewed -
10
Shotgun proteomic analysis of quail liver under feed deprivation and low-energy diet conditions.
Khalifeh DM, Gulyás G, Knop R, Reda GK, Kalló G, Szabó C, Csösz É, Czeglédi L. · 2026-08-11
open access unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.