Affinage

CPT2

Carnitine O-palmitoyltransferase 2, mitochondrial · UniProt P23786

Length
658 aa
Mass
73.8 kDa
Annotated
2026-04-28
100 papers in source corpus 17 papers cited in narrative 17 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

CPT2 is a mitochondrial inner membrane acyltransferase that converts long-chain fatty acyl-carnitines back to fatty acyl-CoA, constituting the terminal step of the carnitine shuttle and a critical gateway for mitochondrial fatty acid β-oxidation in heart, liver, muscle, and immune cells. CPT2 is intrinsically insensitive to malonyl-CoA but acquires malonyl-CoA inhibition when reconstituted with an outer-membrane malonyl-CoA-binding protein (PMID:2401367), and its catalytic activity is regulated by multiple post-translational modifications: Sirt5-mediated desuccinylation at Lys424 and Sirt3-mediated deacetylation activate CPT2 (PMID:38718533, PMID:31866205), whereas SIRT2-mediated deacetylation at Lys239 promotes K48-linked ubiquitination and degradation (PMID:39781464), and HRD1-mediated ubiquitination paradoxically stabilizes the protein (PMID:33207079). At the transcriptional level, CPT2 expression is repressed by E2F2 binding to its promoter and induced by the FAM3A–CaM–FOXA2 signaling axis (PMID:33771899, PMID:35995281). Loss-of-function mutations such as E174K and F383Y cause the muscular and hepatic forms of CPT II deficiency, respectively (PMID:9600456).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 1989 Medium

    Establishing that CPT1 and CPT2 are distinct gene products — enzymatic assays in patients with tissue-specific CPT deficiency demonstrated that CPT2 is a separate enzyme from CPT1, resolving a long-standing question about whether carnitine palmitoyltransferase activity arose from one or two genes.

    Evidence Isotope exchange assays differentiating CPT1 and CPT2 kinetics in muscle and liver biopsies from deficient patients

    PMID:2809620

    Open questions at the time
    • Limited patient numbers from single lab
    • Tissue-specific isoform identity of CPT1 not molecularly resolved in this study
  2. 1990 High

    Determining the basis of malonyl-CoA regulation — reconstitution of purified inner-membrane CPT2 with an outer-membrane malonyl-CoA-binding protein showed that CPT2 itself is malonyl-CoA insensitive and that inhibition requires an outer-membrane component, establishing the two-component model of carnitine shuttle regulation.

    Evidence Biochemical fractionation, cholate extraction, and reconstitution of rat liver mitochondrial membranes with [¹⁴C]malonyl-CoA binding assays

    PMID:2401367

    Open questions at the time
    • Identity of the outer-membrane malonyl-CoA binding protein not molecularly cloned
    • Whether reconstituted regulation is physiologically relevant in intact mitochondria not tested
  3. 1998 High

    Linking specific mutations to CPT II deficiency phenotypes — expression of E174K and F383Y mutants in COS-1 cells showed that each markedly reduces catalytic activity, with genotype–phenotype correlation to muscular versus hepatic disease forms, establishing the molecular basis of CPT II deficiency.

    Evidence Transfection of mutant CPT2 cDNA in COS-1 cells with direct enzymatic activity measurement

    PMID:9600456

    Open questions at the time
    • Structural mechanism by which each mutation impairs catalysis unknown
    • No crystal structure available to explain residue-specific effects
  4. 2018 Medium

    Identifying a pharmacological rescue pathway for CPT2 deficiency — AMPK activation by GSK773 upregulated CPT2 protein via PGC-1α/ROS/p38 MAPK in patient-derived myotubes, correcting fatty acid oxidation flux, showing CPT2 expression is amenable to transcriptional/signaling rescue.

    Evidence Patient-derived CPT2-deficient myotubes treated with AMPK agonist, FAO flux assays, siRNA epistasis

    PMID:30007356

    Open questions at the time
    • In vivo efficacy of AMPK-mediated CPT2 rescue not demonstrated
    • Whether residual mutant CPT2 protein is stabilized or new wild-type-like protein is induced unclear
  5. 2019 High

    Revealing a deacetylation–dimerization activation axis — low-level palmitate triggers a CDK1→SIRT3 cascade that deacetylates and dimerizes CPT2, enhancing fatty acid oxidation, establishing that CPT2 quaternary structure is regulated by acetylation status.

    Evidence In vitro kinase assays, co-IP, SIRT3 phosphorylation and CPT2 acetylation/dimerization assays in liver cells and mouse CCl4 model

    PMID:31866205

    Open questions at the time
    • Specific acetylation sites on CPT2 targeted by SIRT3 in this cascade not mapped
    • Structural basis of how dimerization enhances activity unknown
  6. 2020 Medium

    Uncovering a stabilizing ubiquitination mechanism — HRD1-mediated K48-linked ubiquitination paradoxically stabilizes CPT2 rather than promoting degradation, and its loss under glutamine deprivation destabilizes CPT2 in breast cancer cells, revealing a non-canonical ubiquitin signal.

    Evidence Reciprocal co-IP, ubiquitination assays, HRD1 knockdown/knockout in triple-negative breast cancer cells

    PMID:33207079

    Open questions at the time
    • Mechanism by which K48-ubiquitin stabilizes rather than degrades CPT2 unresolved
    • Not validated outside breast cancer cell lines
  7. 2021 High

    Identifying direct transcriptional repression of CPT2 — ChIP demonstrated E2F2 binds the CPT2 promoter to repress transcription; E2f1/E2f2 knockout mice showed elevated CPT2, increased FAO, and resistance to hepatocarcinogenesis, linking CPT2 transcription to cell-cycle regulators and cancer.

    Evidence ChIP for E2F2 on Cpt2 promoter, E2f1/E2f2 knockout mice, liver-specific knockdown/overexpression, FAO assays

    PMID:33771899

    Open questions at the time
    • Whether E2F2 repression is cell-cycle-phase dependent unknown
    • Other E2F family members' roles at the CPT2 promoter not systematically tested
  8. 2022 Medium

    Defining an upstream signaling cascade for CPT2 induction — FAM3A-released ATP activates P2 receptors, driving CaM nuclear translocation where CaM co-activates FOXA2 to transcribe CPT2 in hepatocytes, establishing a complete extracellular-to-nuclear signaling pathway for CPT2 regulation.

    Evidence RNA-seq, CaM nuclear translocation assays, FOXA2 co-activator assays, FAM3A-deficient hepatocytes and mice

    PMID:35995281

    Open questions at the time
    • Whether FOXA2 directly binds CPT2 promoter elements not shown by ChIP
    • Single-lab observation awaiting replication
  9. 2022 Medium

    Mapping a Sirt3-dependent acetylation site controlling platelet survival — K79 acetylation of CPT2 (accumulating as NAD⁺/Sirt3 activity declines during storage) attenuates fatty acid oxidation, causing acylcarnitine buildup and shortened platelet lifespan, connecting CPT2 post-translational regulation to transfusion biology.

    Evidence Mass spectrometry acetylation site identification, Sirt3 activity assays, pharmacological agonists, in vitro/in vivo platelet survival assays

    PMID:35728063

    Open questions at the time
    • K79 mutagenesis not performed to confirm site specificity
    • Role of other deacetylases at this site not excluded
  10. 2024 High

    Identifying succinylation as a novel inhibitory modification — Sirt5-mediated desuccinylation at Lys424 activates CPT2; loss of Sirt5 in diabetic hearts causes Lys424 succinylation, inactivating CPT2 and impairing FAO, adding a new post-translational regulatory dimension.

    Evidence Succinylomics MS, K424R mutagenesis, enzymatic activity assays, cardiac-specific Sirt5 knockout and overexpression mice

    PMID:38718533

    Open questions at the time
    • Succinyl-CoA-dependent succinylation writer not identified
    • Whether Lys424 succinylation occurs in non-cardiac tissues unknown
  11. 2024 High

    Demonstrating that CPT2-dependent FAO is dispensable for humoral immunity — lymphocyte-specific CPT2 knockout mice showed markedly reduced fatty acid-derived citrate in B cells yet normal B cell development, germinal center reactions, and antibody production, delimiting the tissues in which CPT2 is functionally essential.

    Evidence Lymphocyte-specific CPT2 knockout mice, ¹³C isotope tracing, flow cytometry, in vivo antigen challenge

    PMID:39258879

    Open questions at the time
    • Whether CPT2-independent compensatory FAO pathways exist in B cells not investigated
    • T cell-specific functional consequences not deeply characterized
  12. 2025 High

    Revealing a degradation-promoting deacetylation — SIRT2 deacetylates CPT2 at Lys239, promoting ubiquitination and degradation, thereby suppressing FAO in the diabetic heart — in contrast to SIRT3-mediated deacetylation which activates CPT2, demonstrating sirtuin-specific opposing outcomes.

    Evidence Cardiac-specific SIRT2 overexpression/knockdown in STZ/HFD diabetic mice, K239 mutagenesis, ubiquitination assays

    PMID:39781464

    Open questions at the time
    • The E3 ligase mediating SIRT2-induced CPT2 ubiquitination not identified
    • How K239 deacetylation exposes a degron is structurally unexplained

Open questions

Synthesis pass · forward-looking unresolved questions
  • A high-resolution structure of CPT2 — ideally with bound substrate or acyl-carnitine — is needed to explain how specific lysine modifications (K79, K239, K424) and disease mutations (E174K, F383Y) affect catalysis and dimerization, and how the outer-membrane malonyl-CoA-binding protein confers inhibitor sensitivity.
  • No crystal or cryo-EM structure of mammalian CPT2 published
  • Molecular identity of the outer-membrane malonyl-CoA-binding partner remains unresolved
  • Relative contributions of competing sirtuin pathways in vivo across tissues not integrated

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016740 transferase activity 6
Localization
GO:0005739 mitochondrion 7
Pathway
R-HSA-1430728 Metabolism 7 R-HSA-1643685 Disease 2
Partners
CDK1E2F2FOXA2HRD1MUL1SIRT2SIRT3SIRT5

Evidence

Reading pass · 17 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2024 Sirt5 (a lysine de-succinylase) directly desuccinylates CPT2 at Lys424; succinylation of Lys424 by Sirt5 deficiency inactivates CPT2 enzymatic activity, leading to accumulation of long-chain fatty acyl-carnitines and impaired fatty acid oxidation in the diabetic heart. The CPT2 K424R mutation (mimicking desuccinylation) counteracts this inactivation. Succinylomics mass spectrometry, site-directed mutagenesis (K424R), enzymatic activity assays, cardiac-specific Sirt5 knockout and overexpression mouse models Redox biology High 38718533
2019 Low-level palmitate activates a CDK1-SIRT3-CPT2 cascade in liver cells: CDK1 phosphorylates SIRT3, which in turn deacetylates and dimerizes CPT2, thereby enhancing fatty acid oxidation and mitochondrial homeostasis. In vitro kinase assays, co-immunoprecipitation, SIRT3 phosphorylation and CPT2 acetylation/deacetylation assays, liver cell models, mouse CCl4 hepatotoxicity model Developmental cell High 31866205
2025 SIRT2, localized in cardiac mitochondria, deacetylates CPT2 at K239, which enhances CPT2 ubiquitination and reduces protein stability, thereby inhibiting fatty acid oxidation and ROS production in the diabetic heart. Cardiac-specific SIRT2 overexpression/knockdown mouse models (STZ/HFD), co-immunoprecipitation, ubiquitination assays, site-directed mutagenesis at K239, FAO measurement International journal of biological sciences High 39781464
2020 HRD1 (an E3 ubiquitin ligase) directly ubiquitinates CPT2 via K48-linked ubiquitination, paradoxically stabilizing CPT2 protein; under glutamine deprivation, HRD1 downregulation leads to CPT2 destabilization and impaired fatty acid oxidation in triple-negative breast cancer cells. Co-immunoprecipitation, ubiquitination assays, HRD1 knockdown/knockout, CPT2 inhibition, in vitro and in vivo proliferation assays Molecular oncology Medium 33207079
2025 SLC44A2 (a mitochondrial membrane protein) promotes MUL1 (mitochondrial E3 ubiquitin ligase)-mediated degradation of CPT2 by enhancing the MUL1-CPT2 protein interaction, thereby suppressing fatty acid oxidation and colorectal cancer progression. Co-immunoprecipitation, ubiquitination assays, SLC44A2 overexpression/knockdown, in vitro and in vivo functional assays Cell death & disease Medium 40592838
2022 CPT2 K79 acetylation (driven by NAD+ exhaustion and decreased Sirt3 activity during platelet storage) attenuates CPT2-dependent fatty acid oxidation, causing long-chain acylcarnitine accumulation, mitochondrial damage, and shortened platelet life span. Acetylation site identification by mass spectrometry, Sirt3 activity assays, pharmacological Sirt3 agonists, CPT1/AMPK inhibitors, in vitro and in vivo platelet survival assays Blood advances Medium 35728063
1990 CPT2 (carnitine palmitoyltransferase from the mitochondrial inner membrane) is intrinsically insensitive to malonyl-CoA inhibition; however, when reconstituted with a malonyl-CoA binding protein solubilized from the outer membrane, CPT2 becomes inhibitable by malonyl-CoA, demonstrating that malonyl-CoA sensitivity requires the outer membrane component. Biochemical fractionation of rat liver mitochondrial inner and outer membranes, cholate extraction, CPT activity reconstitution assay, [14C]malonyl-CoA binding FEBS letters High 2401367
1989 CPT1 and CPT2 are distinct enzymes: patients with hepatic CPT1 deficiency in fibroblasts and liver show normal CPT1 and CPT2 activities in muscle under saturating substrate conditions, while muscular-form patients show marked CPT2 deficiency. This establishes that CPT1 and CPT2 are separate proteins and that CPT1 may exist as tissue-specific isoforms. Isotope exchange assay differentiating CPT1 and CPT2 activities in muscle biopsies; kinetic analysis (Km for carnitine and palmitoyl-CoA) in patient and control samples Journal of the neurological sciences Medium 2809620
1998 Two missense mutations in CPT2 (E174K and F383Y) markedly decrease CPT2 catalytic activity; a polymorphism F352C does not alter activity. Genotype-phenotype analysis shows F383Y (homozygous) causes hepatic phenotype and E174K (homozygous) causes muscular phenotype of CPT II deficiency. Transfection of mutant CPT2 cDNA in COS-1 cells, enzymatic activity assay, molecular analysis Human mutation High 9600456
2022 CPT2 mediates fatty acid oxidation in the mitochondria; its inhibition by aminocarnitine during endotoxaemia causes accumulation of long-chain acylcarnitines in the heart, which inhibits pyruvate metabolism and exacerbates inflammation-induced cardiac dysfunction. LPS-induced endotoxaemia mouse model, aminocarnitine pharmacological CPT2 inhibition, mitochondrial respirometry, acylcarnitine metabolite profiling Journal of cellular and molecular medicine Medium 32896106
2021 E2F2 binds directly to the CPT2 promoter and represses its transcription in mouse liver during high-fat diet/DEN-induced hepatocarcinogenesis; E2f1 and E2f2 knockout mice show enhanced CPT2 expression and increased fatty acid oxidation, and are resistant to hepatocarcinogenesis. ChIP showing E2F2 binding to Cpt2 promoter, E2f1/E2f2 knockout mouse models, E2f2 liver-specific knockdown and overexpression, FAO measurement, gene expression analysis Cancer research High 33771899
2022 The FAM3A-ATP-P2 receptor-calmodulin (CaM)-FOXA2-CPT2 pathway regulates fatty acid oxidation in hepatocytes: FAM3A-induced ATP release activates P2 receptors, promoting CaM nuclear translocation where it acts as a co-activator of FOXA2 to drive CPT2 transcription. High-throughput RNA sequencing, CaM nuclear translocation assays, FOXA2 co-activator assays, FAM3A-deficient hepatocytes and mice, pharmacological pathway dissection Metabolism: clinical and experimental Medium 35995281
2025 SLC25A42 (a mitochondrial CoA transporter) promotes acetylation of CPT2, which increases CPT2 expression and enhances fatty acid oxidation; this reprograms lipid metabolism to support gastric cancer growth and ferroptosis resistance. Co-immunoprecipitation, acetylation assays, SLC25A42 knockout/overexpression, FAO measurement, in vitro and in vivo proliferation and ferroptosis assays Cell death & disease Medium 40246810
2024 CPT2-mediated fatty acid oxidation is dispensable for humoral immunity: lymphocyte-specific CPT2 knockout mice show markedly reduced fatty acid-derived citrate production in B cells (confirmed by stable [13C] isotope tracing) but exhibit normal B cell development, activation, germinal center formation, and antibody production. Lymphocyte-specific CPT2 knockout mice, stable [13C] isotope tracing of fatty acid oxidation, flow cytometry, in vivo antigen challenge Journal of immunology High 39258879
2018 AMPK activation by GSK773 upregulates CPT2 protein expression in patient-derived CPT2-deficient myotubes, correcting deficient FAO flux and C16-acylcarnitine accumulation; effects are mediated through PGC-1α, ROS, and p38 MAPK pathways. Patient-derived myotube cultures, FAO flux assays, acylcarnitine profiling, siRNA knockdowns, pharmacological inhibitors, CPT2 protein quantification Human molecular genetics Medium 30007356
2025 CPT2 knockdown in colorectal cancer cells causes accumulation of glycerophospholipids (phosphatidylcholine and phosphatidylethanolamine) via enhanced GPAT4-mediated glycerophospholipid biosynthesis; this promotes autophagosome maturation/elongation and selective autophagy (lipophagy), accelerating tumor progression. CPT2 knockdown, metabolite profiling (glycerophospholipids), transcriptomics, GPAT4 functional analysis, autophagy flux assays, in vitro and in vivo models Communications biology Medium 41107458
2024 GBA3 acts as a transcriptional co-activator to promote CPT2 expression in hepatocytes, thereby increasing fatty acid oxidation, reducing ROS, inhibiting MLKL-mediated necroptosis, and slowing NAFLD progression. GBA3 overexpression in HepG2 cells and rat NAFLD model, Seahorse metabolic assay, western blotting, immunohistochemistry, flow cytometry for ROS and apoptosis Aging Medium 38428407

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2018 Chemoproteomics reveals baicalin activates hepatic CPT1 to ameliorate diet-induced obesity and hepatic steatosis. Proceedings of the National Academy of Sciences of the United States of America 276 29891721
2014 Lipid catabolism via CPT1 as a therapeutic target for prostate cancer. Molecular cancer therapeutics 257 25122071
2023 Mitochondrial morphology controls fatty acid utilization by changing CPT1 sensitivity to malonyl-CoA. The EMBO journal 145 36917141
2004 RORalpha regulates the expression of genes involved in lipid homeostasis in skeletal muscle cells: caveolin-3 and CPT-1 are direct targets of ROR. The Journal of biological chemistry 144 15199055
2012 Increased mitochondrial activity in BMP7-treated brown adipocytes, due to increased CPT1- and CD36-mediated fatty acid uptake. Antioxidants & redox signaling 81 22938691
1987 Mutants of Saccharomyces cerevisiae defective in sn-1,2-diacylglycerol cholinephosphotransferase. Isolation, characterization, and cloning of the CPT1 gene. The Journal of biological chemistry 80 3029130
2022 The activation of PPARγ enhances Treg responses through up-regulating CD36/CPT1-mediated fatty acid oxidation and subsequent N-glycan branching of TβRII/IL-2Rα. Cell communication and signaling : CCS 79 35392915
2020 MSC-induced lncRNA AGAP2-AS1 promotes stemness and trastuzumab resistance through regulating CPT1 expression and fatty acid oxidation in breast cancer. Oncogene 78 33273726
1990 The sn-1,2-diacylglycerol cholinephosphotransferase of Saccharomyces cerevisiae. Nucleotide sequence, transcriptional mapping, and gene product analysis of the CPT1 gene. The Journal of biological chemistry 75 2153142
2021 Targeted Inhibition of LPL/FABP4/CPT1 fatty acid metabolic axis can effectively prevent the progression of nonalcoholic steatohepatitis to liver cancer. International journal of biological sciences 70 34803493
1991 sn-1,2-diacylglycerol choline- and ethanolaminephosphotransferases in Saccharomyces cerevisiae. Nucleotide sequence of the EPT1 gene and comparison of the CPT1 and EPT1 gene products. The Journal of biological chemistry 69 1848238
2024 Sirt5 improves cardiomyocytes fatty acid metabolism and ameliorates cardiac lipotoxicity in diabetic cardiomyopathy via CPT2 de-succinylation. Redox biology 65 38718533
2018 Downregulation of CPT2 promotes tumorigenesis and chemoresistance to cisplatin in hepatocellular carcinoma. OncoTargets and therapy 58 29872321
2021 E2F1 and E2F2-Mediated Repression of CPT2 Establishes a Lipid-Rich Tumor-Promoting Environment. Cancer research 56 33771899
2006 Identification of 16 new disease-causing mutations in the CPT2 gene resulting in carnitine palmitoyltransferase II deficiency. Molecular genetics and metabolism 55 16996287
1991 sn-1,2-diacylglycerol choline- and ethanolaminephosphotransferases in Saccharomyces cerevisiae. Mixed micellar analysis of the CPT1 and EPT1 gene products. The Journal of biological chemistry 55 1847919
2002 Mobilization of the Vibrio pathogenicity island between Vibrio cholerae isolates mediated by CP-T1 generalized transduction. FEMS microbiology letters 52 12351223
2019 Low-Level Saturated Fatty Acid Palmitate Benefits Liver Cells by Boosting Mitochondrial Metabolism via CDK1-SIRT3-CPT2 Cascade. Developmental cell 51 31866205
2011 The effect of clozapine on the AMPK-ACC-CPT1 pathway in the rat frontal cortex. The international journal of neuropsychopharmacology 49 21733226
2002 Palmitate-induced cardiac apoptosis is mediated through CPT-1 but not influenced by glucose and insulin. American journal of physiology. Heart and circulatory physiology 49 11788422
2021 A nanodrug system overexpressed circRNA_0001805 alleviates nonalcoholic fatty liver disease via miR-106a-5p/miR-320a and ABCA1/CPT1 axis. Journal of nanobiotechnology 48 34789275
2018 CPT1 regulates the proliferation of pulmonary artery smooth muscle cells through the AMPK-p53-p21 pathway in pulmonary arterial hypertension. Molecular and cellular biochemistry 48 30511343
1998 Two CPT2 mutations in three Japanese patients with carnitine palmitoyltransferase II deficiency: functional analysis and association with polymorphic haplotypes and two clinical phenotypes. Human mutation 47 9600456
2014 Targeting prostate cancer cell metabolism: impact of hexokinase and CPT-1 enzymes. Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine 44 25501281
1989 Normal muscle CPT1 and CPT2 activities in hepatic presentation patients with CPT1 deficiency in fibroblasts. Tissue specific isoforms of CPT1? Journal of the neurological sciences 44 2809620
2008 CPT2 gene mutations resulting in lethal neonatal or severe infantile carnitine palmitoyltransferase II deficiency. Molecular genetics and metabolism 43 18550408
1994 Studies employing Saccharomyces cerevisiae cpt1 and ept1 null mutants implicate the CPT1 gene in coordinate regulation of phospholipid biosynthesis. The Journal of biological chemistry 43 7961831
2020 HRD1 inhibits fatty acid oxidation and tumorigenesis by ubiquitinating CPT2 in triple-negative breast cancer. Molecular oncology 40 33207079
2022 Downregulation of CPT2 promotes proliferation and inhibits apoptosis through p53 pathway in colorectal cancer. Cellular signalling 37 35108639
2001 Hepatic carnitine palmitoyl transferase 1 (CPT1 A) deficiency in North American Hutterites (Canadian and American): evidence for a founder effect and results of a pilot study on a DNA-based newborn screening program. Molecular genetics and metabolism 35 11350183
2021 Differential contributions of choline phosphotransferases CPT1 and CEPT1 to the biosynthesis of choline phospholipids. Journal of lipid research 32 34331935
2021 Adiponectin ameliorates placental injury in gestational diabetes mice by correcting fatty acid oxidation/peroxide imbalance-induced ferroptosis via restoration of CPT-1 activity. Endocrine 32 34859390
1999 Alternative mechanism of cholera toxin acquisition by Vibrio cholerae: generalized transduction of CTXPhi by bacteriophage CP-T1. Infection and immunity 32 10531246
2021 CPT2 down-regulation promotes tumor growth and metastasis through inducing ROS/NFκB pathway in ovarian cancer. Translational oncology 31 33486313
2021 CPT2 downregulation triggers stemness and oxaliplatin resistance in colorectal cancer via activating the ROS/Wnt/β-catenin-induced glycolytic metabolism. Experimental cell research 31 34688609
2010 HIV-protease inhibitors suppress skeletal muscle fatty acid oxidation by reducing CD36 and CPT1 fatty acid transporters. Biochimica et biophysica acta 31 20117238
2017 Obesity-alleviating potential of asiatic acid and its effects on ACC1, UCP2, and CPT1 mRNA expression in high fat diet-induced obese Sprague-Dawley rats. Molecular and cellular biochemistry 28 28993954
2012 Effect of chronic CPT-1 inhibition on myocardial ischemia-reperfusion injury (I/R) in a model of diet-induced obesity. Cardiovascular drugs and therapy 28 22407171
1984 Vibrio cholerae bacteriophage CP-T1: characterization of bacteriophage DNA and restriction analysis. Journal of virology 28 6328035
2014 Ananas comosus L. Leaf Phenols and p-Coumaric Acid Regulate Liver Fat Metabolism by Upregulating CPT-1 Expression. Evidence-based complementary and alternative medicine : eCAM 27 25197313
2023 CPT2-mediated fatty acid oxidation inhibits tumorigenesis and enhances sorafenib sensitivity via the ROS/PPARγ/NF-κB pathway in clear cell renal cell carcinoma. Cellular signalling 26 37541641
2006 Variants within the muscle and liver isoforms of the carnitine palmitoyltransferase I (CPT1) gene interact with fat intake to modulate indices of obesity in French-Canadians. Journal of molecular medicine (Berlin, Germany) 26 17089095
2022 l-Carnitine therapy improves right heart dysfunction through Cpt1-dependent fatty acid oxidation. Pulmonary circulation 25 35911183
2012 Acetyl-L-carnitine supplementation reverses the age-related decline in carnitine palmitoyltransferase 1 (CPT1) activity in interfibrillar mitochondria without changing the L-carnitine content in the rat heart. Mechanisms of ageing and development 25 22322067
1994 CPT-1 inhibition by etomoxir has a chamber-related action on cardiac sarcoplasmic reticulum and isomyosins. The American journal of physiology 25 7810710
1998 Expression of novel isoforms of carnitine palmitoyltransferase I (CPT-1) generated by alternative splicing of the CPT-ibeta gene. The Biochemical journal 24 9693124
2019 The PPARδ Agonist GW501516 Improves Lipolytic/Lipogenic Balance through CPT1 and PEPCK during the Development of Pre-Implantation Bovine Embryos. International journal of molecular sciences 23 31810173
1998 Chromosomal locations of the mouse fatty acid oxidation genes Cpt1a, Cpt1b, Cpt2, Acadvl, and metabolically related Crat gene. Mammalian genome : official journal of the International Mammalian Genome Society 23 9680378
1990 Carnitine palmitoyltransferase (CPT2) from liver mitochondrial inner membrane becomes inhibitable by malonyl-CoA if reconstituted with outer membrane malonyl-CoA binding protein. FEBS letters 22 2401367
2020 Inhibition of CPT2 exacerbates cardiac dysfunction and inflammation in experimental endotoxaemia. Journal of cellular and molecular medicine 20 32896106
2011 A high-fat diet reduces ceramide synthesis by decreasing adiponectin levels and decreases lipid content by modulating HMG-CoA reductase and CPT-1 mRNA expression in the skin. Molecular nutrition & food research 20 21732532
2010 Aerobic exercise's reversal of insulin resistance by activating AMPKα-ACC-CPT1 signaling in the skeletal muscle of C57BL/6 mice. International journal of sport nutrition and exercise metabolism 20 20975105
2022 Quercetin: a silent retarder of fatty acid oxidation in breast cancer metastasis through steering of mitochondrial CPT1. Breast cancer (Tokyo, Japan) 19 35511410
2021 Carnitine palmitoyltransferase 1 (CPT1) alleviates oxidative stress and apoptosis of hippocampal neuron in response to beta-Amyloid peptide fragment Aβ25-35. Bioengineered 19 34424821
2022 CPT2 K79 acetylation regulates platelet life span. Blood advances 18 35728063
2020 Terpenoids from Resina Commiphora Regulating Lipid Metabolism via Activating PPARα and CPT1 Expression. Organic letters 18 32301324
1985 Bacteriophage CP-T1 of Vibrio cholerae. Identification of the cell surface receptor. European journal of biochemistry 18 4065151
2004 Carnitine palmitoyltransferase-1 (CPT-1) activity stimulation by cerulenin via sympathetic nervous system activation overrides cerulenin's peripheral effect. Endocrinology 17 15044358
2024 Reversing metabolic reprogramming by CPT1 inhibition with etomoxir promotes cardiomyocyte proliferation and heart regeneration via DUSP1 ADP-ribosylation-mediated p38 MAPK phosphorylation. Acta pharmaceutica Sinica. B 16 40041918
2022 Imipramine activates FAM3A-FOXA2-CPT2 pathway to ameliorate hepatic steatosis. Metabolism: clinical and experimental 16 35995281
2021 Recurrent acute necrotizing encephalopathy in a boy with RANBP2 mutation and thermolabile CPT2 variant: The first case of ANE1 in Japan. Brain & development 16 34059398
2022 Atorvastatin ameliorates lipid overload-induced mitochondrial dysfunction and myocardial hypertrophy by decreasing fatty acid oxidation through inactivation of the p-STAT3/CPT1 pathway. Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie 15 36402030
2021 Exogenous L-carnitine ameliorates burn-induced cellular and mitochondrial injury of hepatocytes by restoring CPT1 activity. Nutrition & metabolism 15 34167568
2020 Antiobesity and anti-inflammation effects of Hakka stir-fried tea of different storage years on high-fat diet-induced obese mice model via activating the AMPK/ACC/CPT1 pathway. Food & nutrition research 15 32577118
2018 Fluxomic assay-assisted diagnosis orientation in a cohort of 11 patients with myopathic form of CPT2 deficiency. Molecular genetics and metabolism 15 29478820
2023 L-carnitine alleviates synovitis in knee osteoarthritis by regulating lipid accumulation and mitochondrial function through the AMPK-ACC-CPT1 signaling pathway. Journal of orthopaedic surgery and research 14 37237380
2010 Alternative exon usage in the single CPT1 gene of Drosophila generates functional diversity in the kinetic properties of the enzyme: differential expression of alternatively spliced variants in Drosophila tissues. The Journal of biological chemistry 14 20061394
1995 A 43.5 kb segment of yeast chromosome XIV, which contains MFA2, MEP2, CAP/SRV2, NAM9, FKB1/FPR1/RBP1, MOM22 and CPT1, predicts an adenosine deaminase gene and 14 new open reading frames. Yeast (Chichester, England) 14 8619318
2023 Knockdown of carnitine palmitoyltransferase I (CPT1) reduces fat body lipid mobilization and resistance to starvation in the insect vector Rhodnius prolixus. Frontiers in physiology 13 37469565
2015 The Origin and Diversity of Cpt1 Genes in Vertebrate Species. PloS one 13 26421611
2025 Mitochondrial SIRT2-mediated CPT2 deacetylation prevents diabetic cardiomyopathy by impeding cardiac fatty acid oxidation. International journal of biological sciences 11 39781464
2013 Single nucleotide polymorphism in CPT1B and CPT2 genes and its association with blood carnitine levels in acute myocardial infarction patients. Gene 11 23566841
2020 α-Tocopherol suppresses hepatic steatosis by increasing CPT-1 expression in a mouse model of diet-induced nonalcoholic fatty liver disease. Obesity science & practice 10 33680496
2018 A new AMPK activator, GSK773, corrects fatty acid oxidation and differentiation defect in CPT2-deficient myotubes. Human molecular genetics 10 30007356
2024 CPT2-mediated Fatty Acid Oxidation Is Dispensable for Humoral Immunity. Journal of immunology (Baltimore, Md. : 1950) 9 39258879
2011 Normal Levels of Plasma Free Carnitine and Acylcarnitines in Follow-Up Samples from a Presymptomatic Case of Carnitine Palmitoyl Transferase 1 (CPT1) Deficiency Detected Through Newborn Screening in Denmark. JIMD reports 9 23430868
2025 Constituents-composed Chinese medicine of Dajianzhong Decoction ameliorates Non-alcoholic fatty liver disease via AMPK/PPARα/CPT1 signaling. Phytomedicine : international journal of phytotherapy and phytopharmacology 8 41240545
2024 GBA3 promotes fatty acid oxidation and alleviates non-alcoholic fatty liver by increasing CPT2 transcription. Aging 8 38428407
2022 Activation of EP4 alleviates AKI-to-CKD transition through inducing CPT2-mediated lipophagy in renal macrophages. Frontiers in pharmacology 8 36467025
2007 Identification of the infant-type R631C mutation in patients with the benign muscular form of CPT2 deficiency. Neuromuscular disorders : NMD 8 17651973
2025 SLC25A42 promotes gastric cancer growth by conferring ferroptosis resistance through enhancing CPT2-mediated fatty acid oxidation. Cell death & disease 7 40246810
2016 Coexistence of VHL Disease and CPT2 Deficiency: A Case Report. Cancer research and treatment 7 27034144
2024 pparβ regulates lipid catabolism by mediating acox and cpt-1 genes in Scophthalmus maximus under heat stress. Fish physiology and biochemistry 6 38386263
2023 Design and Synthesis of Novel Indole Ethylamine Derivatives as a Lipid Metabolism Regulator Targeting PPARα/CPT1 in AML12 Cells. Molecules (Basel, Switzerland) 6 38202597
2022 CPT1 Mediated Ionizing Radiation-Induced Intestinal Injury Proliferation via Shifting FAO Metabolism Pathway and Activating the ERK1/2 and JNK Pathway. Radiation research 6 36351324
2013 Skeletal muscle-specific CPT1 deficiency elevates lipotoxic intermediates but preserves insulin sensitivity. Journal of diabetes research 6 24319696
2024 Molecular characterization and transcriptional regulation between PPAR and CPT1 in freshwater bivalve Hyriopsis cumingii. International journal of biological macromolecules 5 39278449
2021 Loss of tumor suppressive properties of lipid metabolism enzyme CPT2 in ovarian carcinoma: Comment on "CPT2 down-regulation promotes tumor growth and metastasis through inducing ROS/NFκB pathway in ovarian cancer" by Zhang et al. Translational oncology 5 33857744
2021 Whole body vibration activates AMPK/CPT1 signaling pathway of skeletal muscle in young and aging mice based on metabolomics study. Endocrine journal 5 34955464
1988 Molecular analysis of the packaging signal in bacteriophage CP-T1 of Vibrio cholerae. Molecular & general genetics : MGG 5 3419420
2025 SLC44A2 negatively regulates mitochondrial fatty acid oxidation to suppress colorectal progression by blocking the MUL1-CPT2 interaction. Cell death & disease 4 40592838
2023 Atomoxetine and Fluoxetine Activate AMPK-ACC-CPT1 Pathway in Human SH-SY5Y and U-87 MG Cells. Psychiatry investigation 4 36990664
2016 Effect of chicken leptin recptor short hairpin RNA on expression of JAK2, STAT3, SOCS3 and CPT1 genes in chicken preadipocytes. Animal science journal = Nihon chikusan Gakkaiho 4 27868312
2025 CPT1 deficiency blocks autophagic flux to promote lipid accumulation induced by co-exposure to polystyrene microplastic and cadmium. Frontiers in pharmacology 3 39834803
2025 Mechanisms of Inonotus obliquus (Fr.) Pilát polysaccharides in ameliorating lipid-induced skeletal muscle insulin resistance via PI3K/AKT and AMPK/ACC1/CPT1 signaling pathways. Journal of ethnopharmacology 3 40348308
2025 Porphyromonas gingivalis extracellular vesicles exacerbated osteoporosis by disrupting osteoblast mitochondrial dynamics and inhibiting Cpt2-regulated fatty acid oxidation. Journal of nanobiotechnology 3 41074134
2025 CPT2 inhibition enhances selective autophagy and proliferation in colorectal cancer via GPAT4-dependent glycerophospholipid biosynthesis. Communications biology 3 41107458
2025 Rewiring lipid Metabolism: The central role of CPT1 in metabolic dysfunction. Progress in biophysics and molecular biology 3 41274422
2024 Inhibition of Crif1 protects fatty acid-induced POMC neuron-like cell-line damage by increasing CPT-1 function. American journal of physiology. Endocrinology and metabolism 3 38597829
2024 Recurrent rhabdomyolysis caused by palmitoyltransferase II (CPT-2) deficiency but complete normal acylcarnitine profile: A patient presentation and review of the literature. Molecular genetics and metabolism reports 3 39429887