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Literature summary extracted from

  • Jitrapakdee, S.; Wallace, J.C.
    Structure, function and regulation of pyruvate carboxylase (1999), Biochem. J., 340, 1-16.
No PubMed abstract available

Activating Compound

EC Number Activating Compound Comment Organism Structure
6.4.1.1 acetyl-CoA activation by short-chain derivatives of CoA Mammalia
6.4.1.1 L-aspartate allosteric activator Saccharomyces cerevisiae
6.4.1.1 palmitoyl-CoA activated by long-chain acyl-CoA derivatives Saccharomyces cerevisiae

Application

EC Number Application Comment Organism
6.4.1.1 medicine pyruvate decarboxylase deficiency is an autosomal, recessively inherited disease, patients have less than 5% of normal enzyme activity. Four single point mutations are responsible for some forms of the disease: V145A, R451C, A610T and M743I Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
6.4.1.1
-
Mammalia

Inhibitors

EC Number Inhibitors Comment Organism Structure
6.4.1.1 2-oxoglutarate
-
Saccharomyces cerevisiae
6.4.1.1 aspartate
-
Saccharomyces cerevisiae

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
6.4.1.1 cytosol
-
Saccharomyces cerevisiae 5829
-
6.4.1.1 mitochondrial inner membrane
-
Mammalia 5743
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
6.4.1.1 ATP + pyruvate + HCO3- Saccharomyces cerevisiae
-
ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- Geobacillus stearothermophilus
-
ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- Methanothermobacter thermautotrophicus
-
ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- Homo sapiens
-
ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- Rhizobium etli essential for normal growth ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- Mammalia role in gluconeogenesis, lipogenesis, biosynthesis of neurotransmitter substances and in glucose-induced insulin secretion by pancreatic islets ADP + phosphate + oxaloacetate
-
r

Organism

EC Number Organism UniProt Comment Textmining
6.4.1.1 Geobacillus stearothermophilus
-
-
-
6.4.1.1 Homo sapiens P11498
-
-
6.4.1.1 Mammalia
-
-
-
6.4.1.1 Methanothermobacter thermautotrophicus
-
-
-
6.4.1.1 Rhizobium etli
-
-
-
6.4.1.1 Saccharomyces cerevisiae
-
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
6.4.1.1 side-chain modification one biotin moiety is covalently attached to the side chain of a lysine residue located near the C-terminus of each protomer Mammalia

Reaction

EC Number Reaction Comment Organism Reaction ID
6.4.1.1 ATP + pyruvate + HCO3- + H+ = ADP + phosphate + oxaloacetate mechanism Mammalia
6.4.1.1 ATP + pyruvate + HCO3- + H+ = ADP + phosphate + oxaloacetate mechanism Saccharomyces cerevisiae
6.4.1.1 ATP + pyruvate + HCO3- + H+ = ADP + phosphate + oxaloacetate mechanism Geobacillus stearothermophilus
6.4.1.1 ATP + pyruvate + HCO3- + H+ = ADP + phosphate + oxaloacetate mechanism Methanothermobacter thermautotrophicus
6.4.1.1 ATP + pyruvate + HCO3- + H+ = ADP + phosphate + oxaloacetate mechanism Rhizobium etli
6.4.1.1 ATP + pyruvate + HCO3- + H+ = ADP + phosphate + oxaloacetate mechanism Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
6.4.1.1 adipose tissue high activity Mammalia
-
6.4.1.1 adrenal gland moderate activity Mammalia
-
6.4.1.1 brain moderate activity Mammalia
-
6.4.1.1 fibroblast low activity Mammalia
-
6.4.1.1 heart moderate activity Mammalia
-
6.4.1.1 kidney high activity Mammalia
-
6.4.1.1 liver high activity Mammalia
-
6.4.1.1 mammary gland high activity Mammalia
-
6.4.1.1 pancreas high activity Mammalia
-
6.4.1.1 white blood cell low activity Mammalia
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
6.4.1.1 ATP + pyruvate + HCO3-
-
Mammalia ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3-
-
Saccharomyces cerevisiae ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3-
-
Geobacillus stearothermophilus ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3-
-
Methanothermobacter thermautotrophicus ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3-
-
Rhizobium etli ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3-
-
Homo sapiens ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- essential for normal growth Rhizobium etli ADP + phosphate + oxaloacetate
-
r
6.4.1.1 ATP + pyruvate + HCO3- role in gluconeogenesis, lipogenesis, biosynthesis of neurotransmitter substances and in glucose-induced insulin secretion by pancreatic islets Mammalia ADP + phosphate + oxaloacetate
-
r

Subunits

EC Number Subunits Comment Organism
6.4.1.1 More each protomer consists of two polypeptide chains, with alpha and beta subunits arranged in an (alphabeta)4 structure Methanothermobacter thermautotrophicus
6.4.1.1 tetramer four identical subunits Mammalia

Cofactor

EC Number Cofactor Comment Organism Structure
6.4.1.1 biotin activates through biotinylation of apoenzyme to holoenzyme Saccharomyces cerevisiae
6.4.1.1 biotin activates through biotinylation of apoenzyme to holoenzyme Geobacillus stearothermophilus
6.4.1.1 biotin activates through biotinylation of apoenzyme to holoenzyme Methanothermobacter thermautotrophicus
6.4.1.1 biotin activates through biotinylation of apoenzyme to holoenzyme Rhizobium etli