|
|
Proteins > Pyruvate carboxylase, mitochondrial (PC)
Pyruvate carboxylase, mitochondrial (PC)
|
UniProt Number:
|
P11498
|
|
Alternate Names:
|
Pyruvic carboxylase, PCB
|
|
Structure and Function:
|
Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin cofactor in the first step and the transfer of this carboxyl group to pyruvate in the second making oxaloacetate. Therefore, pyruvate carboxylase catalyzes the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis. In addition to being a key regulator of gluconeogenesis, pyruvate carboxylase has an anaplerotic role providing oxaloacetate to the TCA cycle when needed.
|
|
Disease Associations:
|
Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia.
|
Monoclonal Antibodies
|
Cat. No. |
Name |
Reactivity |
Apps. |
Amount |
|
MS774 |
PC antibody |
ICC, ICE, IP, FLOW |
human, mouse, rat, bovine |
100 µg |
|
ab123451 |
Anti-PCB antibody [10G7BF7] (ab123451) |
ICE, WB, ICC |
Mouse, Rat, Human |
100 µg |
|
|
Browse Products By:
|
|
|
|
|
|
Product Search:
|
|
|
Related Pathways:
Pyruvate Metabolism: Key Nodes
Gluconeogenesis
|
Subscribe to MitoNews
|
|
|
|
|
|
|
|
