CPT1A

UniProt ID: P50416
Organism: Homo sapiens
Review Status: INITIALIZED
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Gene Description

Carnitine O-palmitoyltransferase 1, liver isoform (CPT1A) is the liver-type isoform of carnitine palmitoyltransferase 1, a multi-pass protein of the mitochondrial outer membrane with a large cytoplasmic C-terminal catalytic domain. It catalyzes the committed, rate-limiting step of long-chain fatty acid entry into mitochondria for beta-oxidation, transferring the acyl group of long-chain acyl-CoA thioesters (e.g. palmitoyl-CoA) onto L-carnitine to form long-chain acylcarnitine plus CoA (EC 2.3.1.21). As the principal regulatory node of the carnitine shuttle, its activity is allosterically inhibited by malonyl-CoA, coupling fatty-acid oxidation to fatty-acid synthesis and nutritional state. Loss-of-function variants cause carnitine palmitoyltransferase 1A deficiency, an autosomal recessive disorder of fatty-acid oxidation presenting with hypoketotic hypoglycemia. Beyond its canonical acyltransferase role, CPT1A has reported secondary activities, including a lysine succinyltransferase activity and a catalysis-independent scaffolding function that recruits ZDHHC4 to promote MAVS palmitoylation and sustain the type I interferon response.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0004095 carnitine O-palmitoyltransferase activity
IBA
GO_REF:0000033
ACCEPT
Summary: Phylogenetic (IBA) assignment of the defining molecular function of CPT1A, transfer of a long-chain acyl group from acyl-CoA to carnitine. This is strongly supported by direct experimental characterization of the human enzyme and is the core function of the gene.
Reason: The carnitine O-palmitoyltransferase activity (EC 2.3.1.21) is the well-established catalytic function of CPT1A and is the correct level of specificity. It is corroborated by multiple experimental annotations in this dataset.
Supporting Evidence:
PMID:14517221
Carnitine palmitoyltransferase 1A (CPT1A) is the
GO:0005739 mitochondrion
IBA
GO_REF:0000033
KEEP AS NON CORE
Summary: Phylogenetic assignment placing CPT1A activity in the mitochondrion. Correct but less specific than the experimentally supported mitochondrial outer membrane localization.
Reason: The mitochondrion is a true but general compartment; the specific and informative localization is the mitochondrial outer membrane (GO:0005741), which is captured by experimental annotations. Retained as non-core parent term.
Supporting Evidence:
PMID:11350182
alters neither mitochondrial targeting nor stability of the CPT1A
GO:0006631 fatty acid metabolic process
IBA
GO_REF:0000033
KEEP AS NON CORE
Summary: Phylogenetic assignment of a general fatty acid metabolic role. True but a broad parent of the more specific beta-oxidation / long-chain fatty acid metabolic terms.
Reason: CPT1A is genuinely involved in fatty acid metabolism, but this term is a general parent; the specific process is long-chain fatty acid import for beta-oxidation (GO:0006635, GO:0001676). Kept as non-core.
Supporting Evidence:
PMID:14517221
key regulatory enzyme of
GO:0009437 carnitine metabolic process
IBA
GO_REF:0000033
ACCEPT
Summary: Phylogenetic assignment of a carnitine metabolic role, consistent with CPT1A producing acylcarnitine from carnitine as part of the carnitine shuttle.
Reason: CPT1A consumes L-carnitine to generate long-chain acylcarnitine, directly participating in carnitine metabolism; supported experimentally (PMID:11350182, IDA in this dataset).
Supporting Evidence:
PMID:14517221
Carnitine palmitoyltransferase 1A (CPT1A) is the
GO:0004095 carnitine O-palmitoyltransferase activity
IEA
GO_REF:0000120
ACCEPT
Summary: Electronic assignment (ARBA/RHEA/EC 2.3.1.21) of the core carnitine O-palmitoyltransferase activity, matching the experimentally established function.
Reason: Correct and specific molecular function, consistent with the RHEA:12661 reaction and EC 2.3.1.21 in the UniProt record and multiple experimental annotations.
Supporting Evidence:
PMID:14517221
Carnitine palmitoyltransferase 1A (CPT1A) is the
GO:0005741 mitochondrial outer membrane
IEA
GO_REF:0000120
ACCEPT
Summary: Electronic assignment of the mitochondrial outer membrane localization, matching the experimentally verified subcellular location of CPT1A.
Reason: The mitochondrial outer membrane is the correct, specific compartment for CPT1A and is supported experimentally (PMID:11350182, PMID:14517221) and by the UniProt SUBCELLULAR LOCATION statement.
Supporting Evidence:
PMID:16651524
an outer mitochondrial membrane enzyme
GO:0006853 carnitine shuttle
IEA
GO_REF:0000117
ACCEPT
Summary: Electronic (ARBA) assignment of the carnitine shuttle process. CPT1A catalyzes the first, committed step of the carnitine shuttle that imports long-chain fatty acids into mitochondria.
Reason: CPT1A performs the rate-limiting acylcarnitine-forming step of the carnitine shuttle; a correct and specific process term. Corroborated by the Reactome TAS annotation to the same term.
Supporting Evidence:
PMID:16651524
controls entry of fatty acids into mitochondria and, thereby, fatty acid oxidation
GO:0016746 acyltransferase activity
IEA
GO_REF:0000002
MARK AS OVER ANNOTATED
Summary: InterPro-based electronic assignment of the general acyltransferase activity (from the carnitine acyltransferase domain IPR000542). This is a broad parent of the specific carnitine O-palmitoyltransferase activity.
Reason: Not wrong, but a general parent of GO:0004095 which is annotated more precisely. The specific carnitine O-palmitoyltransferase activity should be preferred as the informative molecular function.
GO:0001666 response to hypoxia
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer of a rat-ortholog phenotype/expression annotation. Reflects downstream physiology rather than CPT1A's molecular function.
Reason: Response to hypoxia is a plausible physiological context for fatty-acid oxidation regulation but is transferred from the rat ortholog and is not a core molecular activity of CPT1A. Retained as non-core.
GO:0001676 long-chain fatty acid metabolic process
IEA
GO_REF:0000107
ACCEPT
Summary: Electronic assignment of long-chain fatty acid metabolic process, matching the substrate specificity of CPT1A (long-chain acyl-CoA thioesters).
Reason: CPT1A acts specifically on long-chain acyl-CoA substrates to enable their mitochondrial oxidation; this specific process term is well supported (also IDA PMID:11350182 in this dataset).
Supporting Evidence:
PMID:14517221
hepatic long-chain fatty acid beta-oxidation
GO:0006006 glucose metabolic process
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog. Reflects the indirect metabolic influence of fatty-acid oxidation on glucose handling rather than a direct role.
Reason: Any effect on glucose metabolism is downstream/indirect (fatty-acid oxidation spares glucose); not a core CPT1A function. Retained as non-core.
GO:0006631 fatty acid metabolic process
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic assignment of the general fatty acid metabolic process, duplicating the IBA annotation to the same broad parent term.
Reason: True but general; the specific process is long-chain fatty acid import for beta-oxidation. Kept as non-core parent.
GO:0006635 fatty acid beta-oxidation
IEA
GO_REF:0000120
ACCEPT
Summary: Electronic assignment (ARBA/UniPathway UPA00659) of fatty acid beta-oxidation. CPT1A catalyzes the rate-limiting import step required for mitochondrial beta-oxidation of long-chain fatty acids.
Reason: CPT1A is the committed step controlling long-chain fatty-acid entry for beta-oxidation; a correct and central process term, corroborated by TAS (PMID:7892212) and ISS annotations.
Supporting Evidence:
PMID:9691089
decreased rate of fatty acid beta-oxidation
GO:0006641 triglyceride metabolic process
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog. UniProt notes a role in hepatic triglyceride metabolism (by similarity), but this is a downstream/indirect physiological consequence.
Reason: Consistent with a hepatic triglyceride-metabolism role inferred by similarity, but indirect relative to the core acyltransferase function. Retained as non-core.
GO:0007584 response to nutrient
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog capturing nutritional regulation of CPT1A/fatty-acid oxidation.
Reason: Reflects physiological regulation (CPT1A is induced by fatty acids and regulated by nutritional state) rather than a core molecular function. Retained as non-core.
GO:0009410 response to xenobiotic stimulus
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog. Off-context response term with no direct mechanistic link to CPT1A's function established for human.
Reason: Xenobiotic-response annotation transferred from the rat ortholog does not inform CPT1A's molecular function and is a likely over-annotation for the human gene.
GO:0009437 carnitine metabolic process
IEA
GO_REF:0000107
ACCEPT
Summary: Electronic assignment of carnitine metabolic process, duplicating the IBA/IDA annotations. Consistent with CPT1A consuming carnitine to form acylcarnitine.
Reason: CPT1A directly participates in carnitine metabolism as part of the carnitine shuttle; correct process term corroborated by experimental (IDA) evidence.
Supporting Evidence:
PMID:16651524
catalyze acyl transfer from various fatty
GO:0031667 response to nutrient levels
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog capturing regulation by nutritional state (fasting/feeding).
Reason: Reflects physiological regulation of CPT1A rather than its core molecular activity. Retained as non-core.
GO:0032000 positive regulation of fatty acid beta-oxidation
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog. CPT1A is the enzyme that carries out the rate-limiting step of beta-oxidation; framing this as CPT1A positively regulating beta-oxidation is largely self-referential.
Reason: CPT1A executes the committed step of long-chain beta-oxidation (captured by GO:0006635); a separate positive-regulation term for the enzyme regulating the very process it catalyzes is an over-annotation.
GO:0042755 eating behavior
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog. Eating-behavior/energy-homeostasis roles are best established for the brain isoform CPT1C, not the liver CPT1A.
Reason: Feeding-behavior control via malonyl-CoA sensing is chiefly attributed to the brain-specific CPT1C (PMID:16651524); assigning eating behavior to liver CPT1A by ortholog transfer is a likely over-annotation.
Supporting Evidence:
PMID:16651524
CPT1c KO mice have lower body weight and food intake
GO:0042802 identical protein binding
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog of self-association. CPT1A is reported to form homo-oligomers (homohexamer/homotrimer, by similarity), but the bare identical protein binding term is uninformative about function.
Reason: Although CPT1A oligomerizes, identical protein binding is an uninformative binding term that does not describe a molecular function; over-annotation.
GO:0043279 response to alkaloid
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog. Off-context response term with no direct mechanistic link to human CPT1A function.
Reason: Transferred response term that does not inform CPT1A's molecular function; likely over-annotation for the human gene.
GO:0045471 response to ethanol
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog. Ethanol modulates hepatic fatty-acid oxidation, but this is downstream physiology transferred from another species.
Reason: Plausible physiological context for a hepatic fatty-acid-oxidation enzyme but not a core function; retained as non-core.
GO:0046320 regulation of fatty acid oxidation
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog. CPT1A is the principal regulatory point of fatty-acid oxidation (malonyl-CoA-sensitive), consistent with a regulatory role.
Reason: CPT1A is the key regulatory node of fatty-acid oxidation, so a regulatory process annotation is defensible, but the informative statement is its catalytic role in beta-oxidation. Retained as non-core.
Supporting Evidence:
PMID:16651524
Malonyl-CoA is an established inhibitor of
GO:0050796 regulation of insulin secretion
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog. Fatty-acid oxidation influences beta-cell insulin secretion, but this is indirect physiology transferred across species.
Reason: Indirect physiological effect rather than a core CPT1A activity; retained as non-core.
GO:0071398 cellular response to fatty acid
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Electronic transfer from the rat ortholog. CPT1A is transcriptionally up-regulated by fatty acids (UniProt INDUCTION; PMID:16271724), consistent with responding to fatty acids.
Reason: Consistent with fatty-acid induction of CPT1A, but this is a regulatory/response context rather than the core molecular function. Retained as non-core.
GO:0097421 liver regeneration
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog. Highly indirect physiological context with no direct mechanistic tie to CPT1A's molecular function.
Reason: Liver-regeneration annotation transferred from the rat ortholog is far removed from CPT1A's molecular activity; likely over-annotation.
GO:1904772 response to tetrachloromethane
IEA
GO_REF:0000107
MARK AS OVER ANNOTATED
Summary: Electronic transfer from the rat ortholog (a hepatotoxicant-exposure response). Off-context and does not inform CPT1A function.
Reason: Toxicant-response term transferred from the rat ortholog; does not describe CPT1A's molecular function. Likely over-annotation.
GO:0001678 intracellular glucose homeostasis
ISS
GO_REF:0000024
KEEP AS NON CORE
Summary: Sequence-similarity transfer (from mouse Cpt1a) of a glucose-homeostasis role. Reflects the indirect metabolic coupling between fatty-acid oxidation and glucose handling.
Reason: Effect on glucose homeostasis is downstream of the enzyme's fatty-acid-oxidation function rather than a direct activity; retained as non-core.
GO:0006635 fatty acid beta-oxidation
ISS
GO_REF:0000024
ACCEPT
Summary: Sequence-similarity transfer of the fatty acid beta-oxidation process, consistent with CPT1A's role as the committed step of long-chain fatty-acid import for beta-oxidation.
Reason: Correct central process term, corroborated by IEA, TAS (PMID:7892212) and the enzyme's established biology.
Supporting Evidence:
PMID:9691089
decreased rate of fatty acid beta-oxidation
GO:0006853 carnitine shuttle
TAS
Reactome:R-HSA-200425
ACCEPT
Summary: Reactome traceable-author-statement placing CPT1A in the carnitine shuttle pathway, where it catalyzes the initial acylcarnitine-forming step.
Reason: Accurately reflects CPT1A's role as the first, rate-limiting step of the carnitine shuttle that imports long-chain fatty acids into mitochondria.
Supporting Evidence:
PMID:16651524
controls entry of fatty acids into mitochondria and, thereby, fatty acid oxidation
GO:0004095 carnitine O-palmitoyltransferase activity
EXP
PMID:14517221
Functional and structural basis of carnitine palmitoyltransf...
ACCEPT
Summary: Experimental characterization of CPT1A carnitine O-palmitoyltransferase activity, including kinetics and disease-variant analysis in a heterologous yeast expression system.
Reason: Directly supports the core catalytic function of CPT1A; disease-causing variants (e.g. G709E, G710E) abolish this activity, confirming its centrality.
Supporting Evidence:
PMID:14517221
mutations G709E and G710E, which abolish
GO:0005739 mitochondrion
IDA
GO_REF:0000052
KEEP AS NON CORE
Summary: Immunofluorescence-based (HPA) localization of CPT1A to the mitochondrion. Correct but less specific than the mitochondrial outer membrane localization.
Reason: Consistent with CPT1A localization; the more specific and informative compartment is the mitochondrial outer membrane (GO:0005741). Retained as non-core parent.
GO:0004095 carnitine O-palmitoyltransferase activity
EXP
PMID:16651524
The brain-specific carnitine palmitoyltransferase-1c regulat...
ACCEPT
Summary: Experimental comparison showing that CPT1A (liver) catalyzes acyl transfer from fatty acyl-CoAs to carnitine, in contrast to the catalytically inactive brain isoform CPT1C.
Reason: Directly supports CPT1A's carnitine O-palmitoyltransferase activity as its core molecular function.
Supporting Evidence:
PMID:16651524
CPT1a and CPT1b catalyze acyl transfer from various fatty
GO:0005741 mitochondrial outer membrane
EXP
PMID:11350182
Molecular and enzymatic characterization of a unique carniti...
ACCEPT
Summary: Experimental determination of CPT1A subcellular location at the mitochondrial outer membrane (variant characterization also assessed mitochondrial targeting).
Reason: The mitochondrial outer membrane is the specific, correct compartment for CPT1A, consistent with the UniProt SUBCELLULAR LOCATION statement and the two-transmembrane-helix outer-membrane topology.
Supporting Evidence:
PMID:11350182
alters neither mitochondrial targeting nor stability of the CPT1A
GO:0005739 mitochondrion
HTP
PMID:34800366
Quantitative high-confidence human mitochondrial proteome an...
KEEP AS NON CORE
Summary: High-throughput mitochondrial proteome study detecting CPT1A in the mitochondrion. Correct but less specific than the outer-membrane localization.
Reason: Supports mitochondrial localization at low specificity; the informative compartment is the mitochondrial outer membrane. Retained as non-core parent.
GO:0030674 protein-macromolecule adaptor activity
IDA
PMID:38016475
CPT1A induction following epigenetic perturbation promotes M...
ACCEPT
Summary: CPT1A acts as an adaptor/scaffold that recruits the ER-localized palmitoyl transferase ZDHHC4 to MAVS, promoting MAVS Cys79-palmitoylation; a catalytically-dead H473A mutant retains this scaffolding activity, showing it is independent of the acyltransferase activity.
Reason: Directly demonstrated adaptor/scaffold function bridging ZDHHC4 and MAVS. This is a genuine but non-core (moonlighting) molecular function, distinct from the canonical carnitine O-palmitoyltransferase activity.
Supporting Evidence:
PMID:38016475
CPT1A recruits the endoplasmic
GO:0045089 positive regulation of innate immune response
IDA
PMID:38016475
CPT1A induction following epigenetic perturbation promotes M...
ACCEPT
Summary: By recruiting ZDHHC4 to palmitoylate and stabilize MAVS, CPT1A sustains a double-stranded-RNA-activated type I interferon response and potentiates antitumor immunity.
Reason: Experimentally supported role in positively regulating the innate (type I IFN) immune response via MAVS stabilization; a real but non-core process for this metabolic enzyme.
Supporting Evidence:
PMID:38016475
sustains, a double-stranded
GO:0004095 carnitine O-palmitoyltransferase activity
IMP
PMID:14517221
Functional and structural basis of carnitine palmitoyltransf...
ACCEPT
Summary: Mutant-phenotype evidence in which disease-causing missense variants (G709E, G710E) abolish CPT1A carnitine O-palmitoyltransferase activity, demonstrating the wild-type enzyme carries out this reaction.
Reason: Supports the core catalytic function through loss-of-function variant analysis; consistent with the EXP annotation from the same study.
Supporting Evidence:
PMID:14517221
mutations G709E and G710E, which abolish
GO:0004095 carnitine O-palmitoyltransferase activity
IMP
PMID:9691089
Molecular basis of hepatic carnitine palmitoyltransferase I ...
ACCEPT
Summary: Mutant-phenotype evidence in which the disease-causing D454G variant expressed in yeast retained only ~2% of wild-type activity, establishing CPT1A's carnitine O-palmitoyltransferase activity.
Reason: Loss-of-function variant confirms the core catalytic function of CPT1A.
Supporting Evidence:
PMID:9691089
D454G mutation is the disease-causing mutation
GO:0005741 mitochondrial outer membrane
IMP
PMID:14517221
Functional and structural basis of carnitine palmitoyltransf...
ACCEPT
Summary: Variant/expression analysis consistent with CPT1A residing at the mitochondrial outer membrane.
Reason: Supports the specific, correct mitochondrial outer membrane localization, concordant with the EXP and ISS annotations to the same term.
Supporting Evidence:
PMID:16651524
an outer mitochondrial membrane enzyme
GO:0016020 membrane
HDA
PMID:19946888
Defining the membrane proteome of NK cells.
MARK AS OVER ANNOTATED
Summary: High-throughput membrane-proteome detection of CPT1A in an NK-like cell line. Correct at a very general level (CPT1A is a membrane protein) but off-context and uninformative.
Reason: CPT1A is indeed a membrane protein, but membrane is a very general term and this detection comes from an unrelated NK-cell membrane proteomics screen; the informative localization is the mitochondrial outer membrane.
GO:0004095 carnitine O-palmitoyltransferase activity
IDA
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I: ...
ACCEPT
Summary: Characterization of the cloned human liver CPT I cDNA/enzyme, establishing it as a carnitine palmitoyltransferase (EC 2.3.1.21) distinct from CPT II.
Reason: Foundational characterization supporting the core carnitine O-palmitoyltransferase activity of human CPT1A.
Supporting Evidence:
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I
GO:0030855 epithelial cell differentiation
IEP
PMID:21492153
Analysis of proteomic changes induced upon cellular differen...
MARK AS OVER ANNOTATED
Summary: Expression-pattern evidence in which CPT1A was among lipid-metabolism proteins up-regulated during differentiation of the Caco-2 intestinal cell line. This is a correlative association, not a demonstrated role in differentiation.
Reason: The IEP annotation reflects a correlative change in CPT1A abundance during Caco-2 differentiation, not evidence that CPT1A drives epithelial cell differentiation; likely over-annotation.
Supporting Evidence:
PMID:21492153
proteins involved in xenobiotic and drug metabolism as well as in lipid metabolism were upregulated upon cellular differentiation
GO:0005741 mitochondrial outer membrane
TAS
Reactome:R-HSA-200406
ACCEPT
Summary: Reactome traceable-author-statement placing CPT1A at the mitochondrial outer membrane, where it transfers palmitoyl to carnitine.
Reason: Concordant with the experimentally established mitochondrial outer membrane localization; correct and specific.
Supporting Evidence:
PMID:16651524
an outer mitochondrial membrane enzyme
GO:0001676 long-chain fatty acid metabolic process
IDA
PMID:11350182
Molecular and enzymatic characterization of a unique carniti...
ACCEPT
Summary: Direct-assay evidence of CPT1A's role in long-chain fatty acid metabolism, consistent with its substrate specificity for long-chain acyl-CoAs.
Reason: Correct, specific process term supported by direct experimental characterization of the enzyme.
Supporting Evidence:
PMID:11350182
a rare disorder
GO:0004095 carnitine O-palmitoyltransferase activity
IDA
PMID:11350182
Molecular and enzymatic characterization of a unique carniti...
ACCEPT
Summary: Direct kinetic characterization of CPT1A carnitine O-palmitoyltransferase activity (KM/Vmax for carnitine and palmitoyl-CoA), with the G710E variant shown to be catalytically inactive.
Reason: Direct experimental support for the core catalytic function of CPT1A.
Supporting Evidence:
PMID:11350182
CPT1A is totally inactive
GO:0005739 mitochondrion
IDA
PMID:11350182
Molecular and enzymatic characterization of a unique carniti...
KEEP AS NON CORE
Summary: Direct evidence that CPT1A localizes to the mitochondrion (mitochondrial targeting assessed for the G710E variant). Correct but less specific than the outer membrane.
Reason: Supports mitochondrial localization; the informative compartment is the mitochondrial outer membrane (GO:0005741). Retained as non-core parent.
Supporting Evidence:
PMID:11350182
alters neither mitochondrial targeting nor stability of the CPT1A
GO:0005741 mitochondrial outer membrane
ISS
GO_REF:0000024
ACCEPT
Summary: Sequence-similarity transfer (from rat Cpt1a, P32198) of the mitochondrial outer membrane localization, consistent with the human experimental data.
Reason: Correct, specific compartment concordant with the human EXP/IMP annotations.
Supporting Evidence:
PMID:16651524
an outer mitochondrial membrane enzyme
GO:0009437 carnitine metabolic process
IDA
PMID:11350182
Molecular and enzymatic characterization of a unique carniti...
ACCEPT
Summary: Direct-assay evidence for CPT1A's role in carnitine metabolism, consistent with its use of L-carnitine as the acyl acceptor.
Reason: Correct process term supported by direct experimental characterization.
Supporting Evidence:
PMID:11350182
a rare disorder
GO:0004095 carnitine O-palmitoyltransferase activity
TAS
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I: ...
ACCEPT
Summary: Traceable-author-statement citing characterization of the human liver CPT I enzyme (EC 2.3.1.21).
Reason: Supports the core carnitine O-palmitoyltransferase activity of CPT1A.
Supporting Evidence:
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I
GO:0005739 mitochondrion
TAS
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I: ...
KEEP AS NON CORE
Summary: Traceable-author-statement for mitochondrial localization of the human liver CPT I. Correct but general.
Reason: Supports mitochondrial localization at low specificity; outer membrane is the informative compartment. Retained as non-core parent.
Supporting Evidence:
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I
GO:0006635 fatty acid beta-oxidation
TAS
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I: ...
ACCEPT
Summary: Traceable-author-statement placing human liver CPT I in mitochondrial fatty acid beta-oxidation, where it catalyzes the committed import step.
Reason: Correct, central process term consistent with CPT1A biology and corroborated by IEA/ISS annotations.
Supporting Evidence:
PMID:7892212
Human liver mitochondrial carnitine palmitoyltransferase I

Core Functions

Catalyzes the rate-limiting, malonyl-CoA-inhibited transfer of long-chain acyl groups from acyl-CoA thioesters to L-carnitine at the mitochondrial outer membrane, forming long-chain acylcarnitine and CoA and committing long-chain fatty acids to mitochondrial import and beta-oxidation.

Supporting Evidence:
  • PMID:14517221
    Carnitine palmitoyltransferase 1A (CPT1A) is the
  • PMID:16651524
    controls entry of fatty acids into mitochondria and, thereby, fatty acid oxidation

References

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Notes

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