6.4.1.1: pyruvate carboxylase
This is an abbreviated version!
For detailed information about pyruvate carboxylase, go to the full flat file.
Word Map on EC 6.4.1.1
-
6.4.1.1
-
phosphoenolpyruvate
-
gluconeogenesis
-
carboxykinase
-
biotin
-
oxaloacetate
-
acetyl-coa
-
tricarboxylic
-
gluconeogenic
-
anaplerotic
-
malate
-
citrate
-
tca
-
co2
-
malic
-
pepck
-
carboxylases
-
astrocyte
-
glucose-6-phosphatase
-
biotin-dependent
-
acidosis
-
propionyl-coa
-
citric
-
krebs
-
glutamicum
-
bark
-
pine
-
biotin-containing
-
13c-labeled
-
propionyl
-
isotopomer
-
maritime
-
1-13cglucose
-
avidin
-
hyperammonemia
-
fructose-1,6-diphosphatase
-
carboxyltransferase
-
pyrogenic
-
4.1.1.32
-
1,6-bisphosphatase
-
holocarboxylase
-
transcarboxylase
-
biotinidase
-
pyrolytic
-
leigh
-
ureagenesis
-
medicine
-
glucogenic
-
biotechnology
-
penicillinase
-
13c-enriched
-
pinaster
-
synthesis
-
3-methylcrotonyl-coa
- 6.4.1.1
- phosphoenolpyruvate
-
gluconeogenesis
-
carboxykinase
- biotin
- oxaloacetate
- acetyl-coa
-
tricarboxylic
-
gluconeogenic
-
anaplerotic
- malate
- citrate
- tca
- co2
-
malic
- pepck
- carboxylases
- astrocyte
- glucose-6-phosphatase
-
biotin-dependent
- acidosis
- propionyl-coa
-
citric
-
krebs
- glutamicum
-
bark
- pine
-
biotin-containing
-
13c-labeled
-
propionyl
-
isotopomer
-
maritime
-
1-13cglucose
- avidin
- hyperammonemia
-
fructose-1,6-diphosphatase
- carboxyltransferase
-
pyrogenic
-
4.1.1.32
-
1,6-bisphosphatase
- holocarboxylase
- transcarboxylase
- biotinidase
-
pyrolytic
- leigh
-
ureagenesis
- medicine
-
glucogenic
- biotechnology
- penicillinase
-
13c-enriched
- pinaster
- synthesis
- 3-methylcrotonyl-coa
Reaction
Synonyms
Carboxylase, pyruvate, EhPYC1, HpPyc1p, Mfla_1512, MSmeg_2412, PC, Pcase, PCB, PCC, PCx, PYC, Pyc1, Pyc1p, PYC2, pycA, pyruvate carboxylase, pyruvate carboxylase 1, Pyruvic carboxylase, RePC
ECTree
6.4.1.1 pyruvate carboxylaseAdvanced search results
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results (Substrates Products
Substrates Products on EC 6.4.1.1 - pyruvate carboxylase
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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
ADP + carbamoyl phosphate
ATP + ?
-
at 0.3% of the rate obtained from pyruvate-carboxylation
-
-
?
ADP + carbamoylphosphate
?
reverse reaction is assessed by using substrate analogue carbamoylphosphate
-
-
r
ADP + carbamoylphosphate
?
reverse reaction is assessed by using substrate analogue carbamoylphosphate
-
-
r
ATP + pyruvate + HCO3-
?
-
pyruvate carboxylase deficiency in humans causes severe acidosis
-
-
?
ATP + pyruvate + HCO3-
?
Leptosphaeria michotii
-
one of the enzymes of the asparagine-pyruvate pathway
-
-
?
ATP + pyruvate + HCO3-
?
-
the enzyme occupies a strategic position in the intermediary metabolism of numerous tissues. In the gluconeogenic tissues, e.g. liver, kidney, it catalyzes the first step in the synthesis of glucose from pyruvate. During lipogenesis in liver, adipose tissue and mammary gland it participates in the synthesis of acetyl groups and reducing groups for transport from the mitochondria to the cytosol. In yet other tissues it fulfills an anaplerotic function
-
-
?
ATP + pyruvate + HCO3-
?
-
enzyme is necessary for growth in minimal medium with pyruvate as sole carbon source
-
-
?
ATP + pyruvate + HCO3-
?
-
enzyme synthesis is induced by pyruvate and repressed by tricarboxylic acid cycle intermediates
-
-
?
ATP + pyruvate + HCO3-
?
-
meets anaplerotic and/or biosynthetic needs in metabolism
-
-
?
ATP + pyruvate + HCO3-
ADP + oxaloacetate + phosphate
-
-
-
-
?
ATP + pyruvate + HCO3-
ADP + oxaloacetate + phosphate
-
-
-
-
?
ATP + pyruvate + HCO3-
ADP + oxaloacetate + phosphate
-
-
-
-
?
ATP + pyruvate + HCO3-
ADP + oxaloacetate + phosphate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
ATP in form of MgATP2-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
the enzyme catalyzes a pivotal reaction in gluconeogenesis and lipid metabolism in liver, and is a regulatory enzyme in gluconeogenesis that catalyzes the biotin-dependent carboxylation of pyruvate to form oxaloacetate
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
anaplerotic enzyme for growth on carbohydrates, reaction is a major bottleneck for amino acid production
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
role of anaplerosis in the central carbon metabolism and amino acid synthesis
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
cells containing the enzyme yield significantly more cell mass while generating less acetate
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
ATP in form of MgATP2-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
intact enzyme and PC-(BC) chain are able to cleave ATP in the presence of free D-biotin and absence of pyruvate
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
ATP in form of MgATP2-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview. The enzyme is allosterically regulated by acetyl-CoA and aspartate. In addition to de novo fatty acid synthesis, pyruvate carboxylase is also involved in glyceroneogenesis, a pathway for synthesizing glycerol required for fatty acid re-esterification. Physiological functions and regulation, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
the enzyme catalyzes the biotin-dependent production of oxaloacetate and has important roles in gluconeogenesis, lipogenesis, insulin secretion and other cellular processes
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the enzyme plays a pivotal role in intermediary metabolism. It serves a critical anaplerotic function replenishing the Krebs cycle intermediates by catalyzing the conversion of pyruvate to oxaloacetate. In addition, pyruvate carboxylase controls the first step of hepatic gluconeogenesis, and is involved in lipogenesis
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
the overall catalysis by PC proceeds in two steps. First, the biotin carboxylase domain catalyzes the carboxylation of biotin, which is covalently linked to the biotin carboxylaseCP. Bicarbonate donates the carboxyl group, and ATP is hydrolyzed to ADP in this reaction. The carboxytransferase domain then catalyzes the transfer of the activated carboxyl group to pyruvate to produce the oxaloacetate product
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
role in gluconeogenesis, lipogenesis, biosynthesis of neurotransmitter substances and in glucose-induced insulin secretion by pancreatic islets
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview. The enzyme is allosterically regulated by acetyl-CoA and aspartate. In addition to de novo fatty acid synthesis, pyruvate carboxylase is also involved in glyceroneogenesis, a pathway for synthesizing glycerol required for fatty acid re-esterification. Physiological functions and regulation, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
anaplerotic enzyme
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
ATP in form of MgATP2-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
pyruvate carboxylase, and thereby anaplerosis, is crucial for an appropriate rise in the ATP:ADP ratio in response to fuel metabolism
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview. The enzyme is allosterically regulated by acetyl-CoA and aspartate. In addition to de novo fatty acid synthesis, pyruvate carboxylase is also involved in glyceroneogenesis, a pathway for synthesizing glycerol required for fatty acid re-esterification. Physiological functions and regulation, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the enzyme is involved in mitochondrial metabolism
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
ATP in form of MgATP2-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
essential for normal growth
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
r
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
Pyc1 and Pyc2 display different allosteric properties with respect to acetyl CoA activation and aspartate inhibition, with Pyc1 showing a higher degree of cooperativity than Pyc2, even in the absence of aspartate
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview. The enzyme is allosterically regulated by acetyl-CoA and aspartate
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
Pyc1 and Pyc2 display different allosteric properties with respect to acetyl CoA activation and aspartate inhibition, with Pyc1 showing a higher degree of cooperativity than Pyc2, even in the absence of aspartate
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
ATP in form of MgATP2-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3-
ADP + phosphate + oxaloacetate
the enzyme catalyzes the biotin-dependent production of oxaloacetate and has important roles in gluconeogenesis, lipogenesis, and other cellular processes
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + oxaloacetate + phosphate
-
-
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview. The enzyme is allosterically regulated by acetyl-CoA and aspartate. In addition to de novo fatty acid synthesis, pyruvate carboxylase is also involved in glyceroneogenesis, a pathway for synthesizing glycerol required for fatty acid re-esterification. Physiological functions and regulation, overview
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
-
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
involvement of several ionisable residues in both ATP-cleavage and biotin carboxylation
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
Methanococcus sp.
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
the catalyzed anaplerotic reaction is very important replenishing oxaloacetate withdrawn from the tricarboxylic acid cycle for various pivotal biochemical pathways, overview
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
-
-
-
?
ATP + pyruvate + HCO3- + H+
ADP + phosphate + oxaloacetate
-
-
-
-
?
additional information
?
-
-
HCO3--dependent ATP cleavage catalyzed in absence of pyruvate
-
-
?
additional information
?
-
-
ATP cleavage by the recombinantly expressed isolated biotin carboxylase domain, overview
-
-
?
additional information
?
-
-
enzyme deficiency is a rare autosomal recessive disease
-
-
?
additional information
?
-
-
in yeast, two metabolic pathways leading to the production of oxaloacetate are the pyruvate carboxylase-catalysed reaction and the glyoxylate cycle.When yeast is grown on acetate, pyruvate carboxylase-catalysed oxaloacetate formation is repressed but the glyoxylate cycle is active, and vice versa if grown on glucose minimal medium
-
-
?
additional information
?
-
-
starvation enhances pyruvate carboxylase activity, whereas diabetes also increases gluconeogenesis through enhanced uptake of substrate and increased flux through liver pyruvate carboxylase mice
-
-
?
additional information
?
-
-
the enzyme in adipocytes interacts with prohibitin, a protein involved in mitochondrial biogenesis
-
-
?
additional information
?
-
-
protein, but not the enzyme is essential for import and assembly of alcohol oxidase into peroxisomes
-
?
additional information
?
-
-
the transcarboxylase domain of pyruvate carboxylase is essential for assembly of the peroxisomal flavoenzyme alcohol oxidase
-
-
?
additional information
?
-
-
in yeast, two metabolic pathways leading to the production of oxaloacetate are the pyruvate carboxylase-catalysed reaction and the glyoxylate cycle.When yeast is grown on acetate, pyruvate carboxylase-catalysed oxaloacetate formation is repressed but the glyoxylate cycle is active, and vice versa if grown on glucose minimal medium
-
-
?
additional information
?
-
-
the enzyme interacts with the biotin protein ligase or holocarboxylase, EC 6.3.4.15, and is associated with the peroxisomal alcohol oxidase
-
-
?
additional information
?
-
-
protein, but not the enzyme is essential for import and assembly of alcohol oxidase into peroxisomes
-
?
additional information
?
-
-
the enzyme is regulated by a pyruvate carboxylase regulator, encoded by gene PA5437 or pycR, PycR inactivation results in 100 000fold attenuation of virulence in the rat lung in vivo, PycR is a regulator with pleiotropic effects on virulence factors, such as lipase and esterase expression and biofilm formation, which are important for maintenance of Pseudomonas aeruginosa in chronic lung infection, overview
-
-
?
additional information
?
-
beta-cells possess compensatory systems that are activated in response to suppression of pyruvate carboxylase nzyme levels. The compensatory events include allosteric activation of pyruvate carboxylase due to increased levels of acetyl-CoA and enhanced conversion of isocitrate to 2-oxoglutarate and downstream metabolites via the cytosolic, NADP-dependent isocitrate dehydrogenase
-
-
?
additional information
?
-
-
starvation enhances pyruvate carboxylase activity, whereas diabetes also increases gluconeogenesis through enhanced uptake of substrate and increased flux through liver pyruvate carboxylase rats
-
-
?
additional information
?
-
-
2',3'-O-(2,4,6-trinitrophenyl) adenosine 5'-triphosphate is no substrate
-
-
?
additional information
?
-
in the absence of the biotin carboxylase domain, oxamate-stimulated decarboxylation of oxaloacetate, reaction of EC 4.1.1.3, proceeds through a simple ping-pong bi bi mechanism
-
-
?
additional information
?
-
-
in the absence of the biotin carboxylase domain, oxamate-stimulated decarboxylation of oxaloacetate, reaction of EC 4.1.1.3, proceeds through a simple ping-pong bi bi mechanism
-
-
?
additional information
?
-
Rhizobium etli ATCC 51251
in the absence of the biotin carboxylase domain, oxamate-stimulated decarboxylation of oxaloacetate, reaction of EC 4.1.1.3, proceeds through a simple ping-pong bi bi mechanism
-
-
?
additional information
?
-
-
in yeast, two metabolic pathways leading to the production of oxaloacetate are the pyruvate carboxylase-catalysed reaction and the glyoxylate cycle.When yeast is grown on acetate, pyruvate carboxylase-catalysed oxaloacetate formation is repressed but the glyoxylate cycle is active, and vice versa if grown on glucose minimal medium
-
-
?
additional information
?
-
thermodynamic activation parameters for the pyruvate carboxylase-catalyzed carboxylation of pyruvate are largely independent of acetyl-CoA
-
-
?
additional information
?
-
-
thermodynamic activation parameters for the pyruvate carboxylase-catalyzed carboxylation of pyruvate are largely independent of acetyl-CoA
-
-
?
additional information
?
-
thermodynamic activation parameters for the pyruvate carboxylase-catalyzed carboxylation of pyruvate are largely independent of acetyl-CoA
-
-
?
