phosphoenolpyruvate carboxykinase 2 (mitochondrial phosphoenolpyruvate carboxykinase, PEPCK-M, PCK2, PEPCK2)

Function

• carbohydrate biosynthesis; gluconeogenesis
• phosphorylated upon DNA damage, probably by ATM or ATR

GTP + oxaloacetate <--> GDP + phosphoenolpyruvate + CO2

Cofactor: Mn+2 (putative)

Structure

• belongs to the phosphoenolpyruvate carboxykinase [GTP] family

Compartment

mitochondria

Pathology

• defects in PCK2 are the cause of phosphoenolpyruvate carboxykinase deficiency

More general terms

• phosphoenolpyruvate carboxykinase
• mitochondrial protein
• phosphoprotein

Database

• OMIM: https://mirror.omim.org/entry/261650
• UniProt: http://www.uniprot.org/uniprot/Q16822.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=5106