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
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2.3.1.21
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peroxisome
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triglyceride
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proliferator-activated
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acyl-coas
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malonyl-coa
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obesity
-
carboxylase
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adipose
-
high-fat
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acetyl-coa
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beta-oxidation
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cholesterol
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hepatocytes
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cardiac
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palmitate
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sterol
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lipogenesis
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lipoprotein
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steatosis
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lipase
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amp-activated
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adipocytes
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obese
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acyltransferase
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triacylglycerols
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diet-induced
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element-binding
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etomoxir
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leptin
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lipogenic
-
lipolysis
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adiponectin
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pparalpha
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srebp-1c
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medium-chain
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nafld
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nonalcoholic
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ketogenesis
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stearoyl-coa
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protein-1c
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hfd-induced
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rhabdomyolysis
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palmitate-induced
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srebf1
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anti-obesity
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muscle-type
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myoglobinuria
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lcfas
-
synthesis
-
medicine
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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
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results (Natural Substrates Products
Natural Substrates Products on EC 2.3.1.21 - carnitine O-palmitoyltransferase
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NATURAL SUBSTRATE 
NATURAL 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
palmitoyl-CoA + L-carnitine
L-palmitoylcarnitine + CoA
-
forward reaction by CPT I and reverse reaction by CPT II
-
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
-
enzyme has a key function in regulation of fatty acid beta-oxidation
-
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 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
-
?
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
-
-
-
?
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
-
regulation, mechanism, overview
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
-
?
palmitoyl-CoA + L-carnitine
CoA + L-palmitoylcarnitine
-
-
-
?
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
-
-
-
?
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
?
-
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
?
-
-
CPT1c is necessary for the regulation of energy homeostasis, and does not catalyze acyl transfer from various fatty acyl-CoAs to carnitine
-
-
?
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
-
-
?
