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MS
rate-limiting unreviewed

Carnitine palmitoyltransferase I (CPT-1)

1 reactions · 1 pathways

Clinical / pharmacological. CPT-I deficiency: hypoketotic hypoglycaemia, hepatomegaly

What it does, reaction by reaction

1 reactions

Beta-Oxidation of Fatty Acids Lipid Metabolism · Mitochondrial matrix (peroxisome for VLCFA)

step 2 Irreversible/directional rate-limiting

Fatty acyl-CoA + carnitine → acylcarnitine + CoA-SH

Converts Fatty acyl-CoA Carnitine into Acylcarnitine CoA-SH

› Notes

Carnitine palmitoyltransferase I (CPT-1), located on the outer mitochondrial membrane, transfers the acyl group to carnitine. This is the principal rate-limiting/regulatory step for mitochondrial oxidation of long-chain fatty acids and is inhibited by malonyl-CoA.

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

Europe PMC · fetched just now · sorted by publication date

  1. 1
  2. 2
    Metabolic reprogramming in colorectal cancer: Mechanisms and therapeutic insights.

    Xiao W, Tan D, Zeng W, Nie X, Wang M. · 2026-01-28

    open access unreviewed
  3. 3
    Altered Acylcarnitine Metabolism in cardiac diseases: From molecular mechanisms to translational potential.

    Wu J, Zhang Y, Liu J, Zhao W, Liu X, Dan J. · 2026-09-25

    open access unreviewed
  4. 4
  5. 5
  6. 6
    Tissue-specific transcriptomic responses and skeletal-muscle ultrastructural observations in Semah mahseer (<i>Tor douronensis</i>) under acute thermal and hypoxic conditions.

    Uchuwittayakul A, Phuthong W, Kong C, Rajitdumrong C, Adisornprasert Y, Kumwan B, Meachasompop P, Ely SSP, Va… · 2026-08-22

    open access unreviewed
  7. 7
    <i>Bacteroides cellulosilyticus</i>-derived 2-hydroxyphenylacetic acid rectifies hepatic lipid homeostasis in MASLD by targeting the PPARγ-CD36 axis.

    Chen K, Yang Z, Peng J, Liu C, Yu Y, Cai X, Liu B, Li S, Chen T, Jung S, Tian Y, Xu Q, Rao X, Wu Z, Wang H, D… · 2026-09-01

    open access unreviewed
  8. 8
    Natural products against renal fibrosis: molecular mechanisms, safety concerns, and translational perspectives.

    Liu Y, Gao L, Xu H, Zhou Y, Chen Y, Wang L, Zhao F, Pan T, Liu Z. · 2026-08-24

    open access unreviewed
  9. 9
  10. 10
    Effects of phytosterols supplementation on hepatic lipid metabolism and metabolic outcomes in obese rodent models: a systematic review and meta-analysis.

    Siqueira JS, da Cruz LL, Palacio TLN, Cruzeiro J, Francisqueti-Ferron FV, Aldini G, D'Amato A, Corrêa CR. · 2026-08-27

    unreviewed

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