pyruvate dehydrogenase phosphatase catalytic subunit 1; [pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial; PDP 1; PDPC 1 (PDP1, PPM2C)

Function

• catalyzes dephosphorylation & concomitant reactivation of pyruvate dehydrogenase E1-alpha (putative)
• component of pyruvate dehydrogenase phosphatase, heterodimer of an enzyme catalytic subunit (PDP1) & an enzyme regulatory subunit (PDPR)

[pyruvate dehydrogenase (acetyl-transferring)]phosphate + H2O = [pyruvate dehydrogenase (acetyl-transferring)] + phosphate

Cofactor: binds 2 Mg+2 per subunit (putative)

Structure

belongs to the PP2C family

Compartment

mitochondrial matrix (putative)

Pathology

• defects in PDP1 are the cause of pyruvate dehydrogenase phosphatase deficiency

More general terms

• pyruvate dehydrogenase phosphatase catalytic subunit

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=54704
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:54704
• OMIM: https://mirror.omim.org/entry/605993
• OMIM: https://mirror.omim.org/entry/608782
• UniProt: http://www.uniprot.org/uniprot/Q9P0J1.html