2.3.1.21: carnitine O-palmitoyltransferase
This is an abbreviated version!
For detailed information about carnitine O-palmitoyltransferase, go to the full flat file.
Word Map on EC 2.3.1.21
-
2.3.1.21
-
peroxisome
-
triglyceride
-
proliferator-activated
-
acyl-coas
-
malonyl-coa
-
obesity
-
carboxylase
-
adipose
-
high-fat
-
acetyl-coa
-
beta-oxidation
-
cholesterol
-
hepatocytes
-
cardiac
-
palmitate
-
sterol
-
lipogenesis
-
lipoprotein
-
steatosis
-
lipase
-
amp-activated
-
adipocytes
-
obese
-
acyltransferase
-
triacylglycerols
-
diet-induced
-
element-binding
-
etomoxir
-
leptin
-
lipogenic
-
lipolysis
-
adiponectin
-
pparalpha
-
srebp-1c
-
medium-chain
-
nafld
-
nonalcoholic
-
ketogenesis
-
stearoyl-coa
-
protein-1c
-
hfd-induced
-
rhabdomyolysis
-
palmitate-induced
-
srebf1
-
anti-obesity
-
muscle-type
-
myoglobinuria
-
lcfas
-
synthesis
-
medicine
-
vlcad
-
acaca
-
pharmacology
- 2.3.1.21
- peroxisome
- triglyceride
-
proliferator-activated
- acyl-coas
- malonyl-coa
- obesity
- carboxylase
- adipose
-
high-fat
- acetyl-coa
-
beta-oxidation
- cholesterol
- hepatocytes
- cardiac
- palmitate
- sterol
-
lipogenesis
- lipoprotein
- steatosis
- lipase
-
amp-activated
- adipocytes
-
obese
- acyltransferase
- triacylglycerols
-
diet-induced
-
element-binding
- etomoxir
- leptin
-
lipogenic
-
lipolysis
- adiponectin
- pparalpha
- srebp-1c
-
medium-chain
- nafld
-
nonalcoholic
-
ketogenesis
- stearoyl-coa
- protein-1c
-
hfd-induced
- rhabdomyolysis
-
palmitate-induced
-
srebf1
-
anti-obesity
-
muscle-type
- myoglobinuria
-
lcfas
- synthesis
- medicine
- vlcad
- acaca
- pharmacology
Reaction
Synonyms
acylcarnitine transferase, carnitine O-palmitoyltransferase, carnitine O-palmitoyltransferase I, carnitine palmitoyl transferase 1, carnitine palmitoyl transferase 1A, carnitine palmitoyl transferase-1A, carnitine palmitoyl transferase-I, carnitine palmitoyl-transferase-1c, carnitine palmitoyltransferae 1A, carnitine palmitoyltransferase, carnitine palmitoyltransferase 1, carnitine palmitoyltransferase 1A, carnitine palmitoyltransferase 1B, carnitine palmitoyltransferase 2, carnitine palmitoyltransferase I, carnitine palmitoyltransferase IA, carnitine palmitoyltransferase II, carnitine palmitoyltransferase-1, carnitine palmitoyltransferase-1c, carnitine palmitoyltransferase-A, carnitine palmitoyltransferase-I, carnitine palmitoyltransferases 2, CPT, CPT I, CPT IA, CPT Ialpha1a, CPT Ialpha1b, CPT Ialpha2a, CPT Ibeta, CPT II, CPT-1, CPT-2, CPT-A, CPT-B, CPT-Ialpha, CPT-IL, CPT1, CPT1-A, CPT1-B, CPT1-C, CPT1A, CPT1B, CPT1c, CPT2, CPTi, CPTI beta, CPTII, CPTo, L-carnitine palmitoyltransferase, L-CPT 1, L-CPT1, M-CPT 1, M-CPTI, muscle carnitine palmitoyltransferase I, palmitoylcarnitine transferase, palmitoyltransferase, carnitine
ECTree
2.3.1.21 carnitine O-palmitoyltransferaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 2.3.1.21 - carnitine O-palmitoyltransferase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
4,8-dimethylnonanoyl-CoA + L-carnitine
CoA + 4,8-dimethylnonanoyl-L-carnitine
-
-
-
?
docosanhexaenoyl-CoA + L-carnitine
CoA + L-docosanhexaenoylcarnitine
-
-
-
-
?
palmitoleoyl-CoA + L-carnitine
CoA + L-palmitoleoylcarnitine
-
activity is 47% higher than with palmitoyl-CoA
-
-
?
palmitoyl-CoA + L-carnitine
L-palmitoylcarnitine + CoA
-
forward reaction by CPT I and reverse reaction by CPT II
-
r
trans-2-hexadecenoyl-CoA + L-carnitine
CoA + trans-2-hexadecenoyl-L-carnitine
-
-
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
-
reverse reaction with palmitoylcarnitine, reverse reaction: myristoylcarnitine
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity
-
?, r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
effect of octylglucoside and Triton X-100 on specificity
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity
reverse reaction: acyl-CoA substrates are C4 to C18 acylcarnitines
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
C4 to C18 acyl-CoAs
reverse reaction: acyl-CoA substrates are C4 to C18 acylcarnitines
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I activity is involved in cardiac mitochondrial beta-oxidation flux control
-
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
enzyme has a key function in regulation of fatty acid beta-oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
enzyme has a key function in regulation of fatty acid beta-oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I, involved in regulation of fatty acid oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity
-
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
preference for desaturated long-chain acyl CoAs
-
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
enzyme has a key function in regulation of fatty acid beta-oxidation
-
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity
-
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT II shows greater chain-length substrate specificity for transfer of long chain fatty acyl groups from (-)-acylcarnitine derivatives to CoA-SH with greatest activity being obtained in the transfer of stearoyl and palmitoyl groups
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
overview: activity towards long-chain polyunsaturated fatty acids and their CoA esters
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT II requires preincubation with CoA-SH for manifestation of catalytic activity
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
isozyme CPT II catalyzes the reaction only in the direction towards long-chain fatty acyl-CoA formation, CPT I catalyzes reaction in both directions
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I unlike CPT II requires membrane integrity for catalytic function
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
activity with different substrates of wild-type CPT I with and without gamma-linoleic acid treatment
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I activity is involved in cardiac mitochondrial beta-oxidation flux control
-
r
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
enzyme is involved in apoptosis induction
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I is the rate-limiting enzyme in beta-oxidation of long-chain fatty acids
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
enzyme has a key function in regulation of fatty acid beta-oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
enzyme has a key function in regulation of fatty acid beta-oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I, involved in regulation of fatty acid oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I, involved in regulation of fatty acid oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
CPT I, involved in regulation of fatty acid oxidation
-
?
acyl-CoA + L-carnitine
CoA + L-acylcarnitine
-
substrate specificity at early developmental stages in skeletal muscle
-
?
decanoyl-CoA + L-carnitine
CoA + L-decanoylcarnitine
-
best substrate
-
?
linoleoyl-CoA + L-carnitine
CoA + L-linoleoylcarnitine
-
preference for
-
r
oleoyl-CoA + L-carnitine
CoA + L-oleoylcarnitine
-
activity is 264% higher than with stearoyl-CoA
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
CPT I catalyzes the conversion of long-chain fatty acyl-CoAs to acyl carnitines in the presence of L-carnitine, a rate-limiting step in the transport of long-chain fatty acids from the cytoplasm to the mitochondrial matrix
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
CPT I is considered the rate-limiting step in the oxidation of long-chain fatty acids and is an important site in the regulation of flux through beta-oxidation
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
enzyme inhibition reduces hepatic glucose production and plasma lipids in non-insulin-dependent diabetes mellitus, overview
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
regulation, mechanism, overview
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
the enzyme is important in mitochondrial fatty acid beta-oxidation, overview
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
the enzyme is the main regulatory enzyme involved in fatty acid oxidation
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
deuterium-labeled L-carnitine substrate
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
the enzyme catalyzes the transfer of an acyl-CoA moiety from a long-chain acyl-CoA ester to carnitine to form acylcarnitine
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
regulation, mechanism, overview
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
the enzyme is the main regulatory enzyme involved in fatty acid oxidation
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
deuterium-labeled L-carnitine substrate
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
regulation, mechanism, overview
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
486309, 486551, 486558, 486560, 486562, 486568, 486569, 486570, 486573, 486574, 486580, 486582, 486589, 486591, 673216, 675740, 686125, 686180, 687793, 688449, 688981, 735458, 736793
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
-
r
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
chimeric L-CPT I with exchanged C-terminals between pig and rat enzymes
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
the enzyme is the main regulatory enzyme involved in fatty acid oxidation
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
deuterium-labeled L-carnitine substrate
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
chimeric L-CPT I with exchanged C-terminals between pig and rat enzymes
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
?
additional information
?
-
-
participates in fatty acyl group transport into mitochondria
-
-
?
additional information
?
-
-
participates in fatty acyl group transport into mitochondria
-
-
?
additional information
?
-
-
responsible for transferase activity on the outer side of the inner mitochondrial membrane
-
-
?
additional information
?
-
-
CPT1-A defificiency presents as recurrent attacks of fasting hypoketotic hypoglycemia
-
-
?
additional information
?
-
CPT1-A defificiency presents as recurrent attacks of fasting hypoketotic hypoglycemia
-
-
?
additional information
?
-
CPT1-A defificiency presents as recurrent attacks of fasting hypoketotic hypoglycemia
-
-
?
additional information
?
-
-
the adult form of CPT2 deficiency is characterized by episodes of rhabdomyolysis triggered by prolonged exercise. The prevalent S113L mutation is found in about 50% of mutant alleles. The infantile-type CPT2 deficiency presents as severe attacks of hypoketptic hypoglycemia, occasionally associated with cardiac damage commonly responsible for sudden death before 1 year of age. In addition to theses symptoms features of brain and kidney dysorganogenesis are frequently seen in the neonatal-onset CPT2 deficiency, almost always lethal during the first month of life. 40 mutations habe been characterized in patients with the adult, infantile or neonatal form of CPT2 deficiency
-
-
?
additional information
?
-
the adult form of CPT2 deficiency is characterized by episodes of rhabdomyolysis triggered by prolonged exercise. The prevalent S113L mutation is found in about 50% of mutant alleles. The infantile-type CPT2 deficiency presents as severe attacks of hypoketptic hypoglycemia, occasionally associated with cardiac damage commonly responsible for sudden death before 1 year of age. In addition to theses symptoms features of brain and kidney dysorganogenesis are frequently seen in the neonatal-onset CPT2 deficiency, almost always lethal during the first month of life. 40 mutations habe been characterized in patients with the adult, infantile or neonatal form of CPT2 deficiency
-
-
?
additional information
?
-
the adult form of CPT2 deficiency is characterized by episodes of rhabdomyolysis triggered by prolonged exercise. The prevalent S113L mutation is found in about 50% of mutant alleles. The infantile-type CPT2 deficiency presents as severe attacks of hypoketptic hypoglycemia, occasionally associated with cardiac damage commonly responsible for sudden death before 1 year of age. In addition to theses symptoms features of brain and kidney dysorganogenesis are frequently seen in the neonatal-onset CPT2 deficiency, almost always lethal during the first month of life. 40 mutations habe been characterized in patients with the adult, infantile or neonatal form of CPT2 deficiency
-
-
?
additional information
?
-
-
CPT2 deficiency and glutaric aciduria type 2 are related
-
-
?
additional information
?
-
-
the enzyme is rate-limiting in the mitochondrial fatty axid oxidation pathway, physiologic regulation, overview
-
-
?
additional information
?
-
isoform CPT2 is active with medium and long-chain acyl-CoA esters, whereas virtually no activity is found with short- and very long-chain acyl-CoAs or with branched-chain amino acid oxidation intermediates. Trans-2-enoyl-CoA intermediates are poor substrates, too. Isoform CPT2 is able to reverse its physiological mechanism for medium and long-chain acyl-CoAs contributing to the abnormal acylcarnitines profiles characteristic of most mitochondrial fatty acid beta-oxidation disorders
-
-
?
additional information
?
-
-
isoform CPT2 is active with medium and long-chain acyl-CoA esters, whereas virtually no activity is found with short- and very long-chain acyl-CoAs or with branched-chain amino acid oxidation intermediates. Trans-2-enoyl-CoA intermediates are poor substrates, too. Isoform CPT2 is able to reverse its physiological mechanism for medium and long-chain acyl-CoAs contributing to the abnormal acylcarnitines profiles characteristic of most mitochondrial fatty acid beta-oxidation disorders
-
-
?
additional information
?
-
no substrates are: isovaleryl-CoA, isobutyryl-CoA, acetoacetyl-CoA, DL-3-hydroxybutyryl-CoA, 3-hydroxyisobutyryl-CoA, 2-methylacetoacetyl-CoA, methylcrotonyl-CoA, glutaryl-CoA, 3-hydroxy-3-methylglutaryl-CoA, 2-methylbutyryl-CoA, 2-methyl-3-hydroxybutyryl-CoA
-
-
?
additional information
?
-
-
no substrates are: isovaleryl-CoA, isobutyryl-CoA, acetoacetyl-CoA, DL-3-hydroxybutyryl-CoA, 3-hydroxyisobutyryl-CoA, 2-methylacetoacetyl-CoA, methylcrotonyl-CoA, glutaryl-CoA, 3-hydroxy-3-methylglutaryl-CoA, 2-methylbutyryl-CoA, 2-methyl-3-hydroxybutyryl-CoA
-
-
?
additional information
?
-
the enzyme is inactive with short-chain acyl-CoAs and intermediates of the branched-chain amino acid oxidation pathway
-
-
?
additional information
?
-
-
the enzyme is inactive with short-chain acyl-CoAs and intermediates of the branched-chain amino acid oxidation pathway
-
-
?
additional information
?
-
no activity with 2,6-dimethylheptanoyl-CoA
-
-
?
additional information
?
-
-
CPT1c is necessary for the regulation of energy homeostasis, and does not catalyze acyl transfer from various fatty acyl-CoAs to carnitine
-
-
?
additional information
?
-
-
the brain-specific isozyme CPT1c has a unique function or activation mechanism, and does not catalyze acyl transfer from various fatty acyl-CoAs to carnitine, no activity with diverse acyl-CoAs, overview
-
-
?
additional information
?
-
-
the branched chain fatty acid CoA-thioesters pristanoyl-CoA and phytanoyl-CoA, i.e. 2,6,10,14-tetramethylhexadecanoyl-CoA and 3,7,11,15-tetramethylhexadecanoyl-CoA, are no substrates in vivo, since they cannot be transported into mitochondria of liver
-
-
?
additional information
?
-
-
acyl-CoA binding protein ACBP forms binary complexes with acyl-CoA and possibly transfers acyl-CoA to CPT I, CPT I induces conformational changes in ACBP leading to release of acyl-CoA
-
-
?
additional information
?
-
-
participates in fatty acyl group transport into mitochondria
-
-
?
additional information
?
-
-
gamma-linolenic acid diet leads to several physiological effects during cancer therapy, e.g. reduction of mitochondrial metabolic activity, modification of mitochondrial outer membrane composition, increase in lipid peroxidation, cytoplasmic accumulation of acyl-CoA, reduction of CPT I activity, and malonyl-CoA sensitivity
-
-
?
additional information
?
-
-
the enzyme is involved in long-chain fatty acid oxidation, overview
-
-
?
additional information
?
-
-
the enzyme is rate-limiting in the mitochondrial fatty axid oxidation pathway, physiologic regulation, inhibition of fatty acid oxidation increases food intake, overview
-
-
?
additional information
?
-
-
activity with unsaturated fatty acyl-CoA substrates is significantly higher or trended higher than that with their saturated counterparts of the same chain length, and activity is higher with shorter fatty acyl-CoAs for a given number of double bonds. The pattern of substrate preference is not different between captive white-throated sparrows in a migratory or non-migratory state
-
-
?
