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Literature summary for 6.4.1.1 extracted from

  • Wallace, J.
    My favorite pyruvate carboxylase (2010), IUBMB Life, 62, 535-538.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 6.4.1.1

Activating Compound

Activating Compound Comment Organism Structure
acetyl-CoA
-
 Homo sapiens
acetyl-CoA
-
 Rattus norvegicus

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+ required for activity Homo sapiens
Mg2+ required for activity Rattus norvegicus

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-
Rattus norvegicus
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + pyruvate + HCO3- + H+
-
 Homo sapiens ADP + oxaloacetate + phosphate
-
 r
ATP + pyruvate + HCO3- + H+
-
 Rattus norvegicus ADP + oxaloacetate + phosphate
-
 r

Subunits

Subunits Comment Organism
homotetramer
-
 Homo sapiens
homotetramer
-
 Rattus norvegicus

Cofactor

Cofactor Comment Organism Structure
ATP
-
 Homo sapiens
ATP
-
 Rattus norvegicus
biotin
-
 Homo sapiens
biotin
-
 Rattus norvegicus

Expression

Organism Comment Expression
Rattus norvegicus expression of pyruvate carboxylase is be increased 2-5fold at the onset of obesity in Zucker fatty rats up

General Information

General Information Comment Organism
malfunction pyruvate decarboxylase deficiency type A is characterised by hypoglycemia accompanied by mild to moderate lactic acidemia and sometimes elevated ketone body levels. Type B, having no detectable pyruvate carboxylase protein in any tissues, is the most severe form which leads to death generally within three months from lactic academia accompanied by hyperammonemia, citrullinemia and hyperlysinemia. Type C has a benign phenotype associated with episodes of lactic acidemia and no psychomotor disorders Homo sapiens