Loading the page.
Proteins/UNIPROT:P50416
Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Also possesses a lysine succinyltransferase activity that can regulate enzymatic activity of substrate proteins such as ENO1 and metabolism independent of its classical carnitine O-palmitoyltransferase activity. Plays an important role in hepatic triglyceride metabolism (By similarity). Also plays a role in inducible regulatory T-cell (iTreg) differentiation once activated by butyryl-CoA that antagonizes malonyl-CoA-mediated CPT1A repression (By similarity). Sustains the IFN-I response by recruiting ZDHCC4 to palmitoylate MAVS at the mitochondria leading to MAVS stabilization and activation. Promotes ROS-induced oxidative stress in liver injury via modulation of NFE2L2 and NLRP3-mediated signaling pathways (By similarity)..
Function from UniProtKB (P50416), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 1 · substrate of
1 compound
1 compound.
0 reported routes1 assembled route
1 row
383 statements
383 statements · 8 reference databases · Subject: CPT1A · Sources: Bgee, gene ontology, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, WikiPathways · Rows fetched: 300
106 reactions
(2E)-hexadecenoyl-CoA+(S)-carnitineCHEBI:1146900+MAM02117c
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR00103CC BY 4.0Reference data
(S)-carnitine+oleoyl-CoACHEBI:1146900+MAM02639c
7 names
Resolves to: CPT1A
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR00108CC BY 4.0Reference data
butanoyl-CoA+(S)-carnitineCHEBI:1146900+MAM02635c
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR00159CC BY 4.0Reference data
(2E)-octadecenoyl-CoA+(S)-carnitineMAM00126c+CHEBI:1146900
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR00452CC BY 4.0Reference data
(S)-carnitine+(9Z,12Z,15Z)-octadecatrienoyl-CoACHEBI:1146900+MAM03727c
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR01488CC BY 4.0Reference data
(S)-carnitine+palmitoyl-CoACHEBI:1146900+O-hexadecanoyl-(R)-carnitine
Reaction pageReversibleBalancedEC 2.3.1.21peroxisome
Human-GEM MAR02121CC BY 4.0Reference data
(S)-carnitine+(9Z,12Z)-octadecadienoyl-CoACHEBI:1146900+MAM02388x
Reaction pageLeft to rightBalancedEC 2.3.1.21peroxisome
Human-GEM MAR02122CC BY 4.0Reference data
arachidonoyl-CoA+(S)-carnitineMAM01363x+CHEBI:1146900
Reaction pageLeft to rightBalancedEC 2.3.1.21peroxisome
Human-GEM MAR02123CC BY 4.0Reference data
CHEBI:1146900+O-acetyl-(R)-carnitineCHEBI:15351+(S)-carnitine
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR02591CC BY 4.0Reference data
CHEBI:1146900+MAM02657c(S)-carnitine+propionyl-CoA
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR02594CC BY 4.0Reference data
CHEBI:1146900+MAM02443c(S)-carnitine+malonyl-CoA
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR02599CC BY 4.0Reference data
CHEBI:1146900+O-dodecanoyl-R-carnitinedodecanoyl-CoA+(S)-carnitine
Catalysed by CPT1A or CPT1C or CPT1B
Reaction pageReversibleBalancedEC 2.3.1.21cytosol
Human-GEM MAR02602CC BY 4.0Reference data