background:
PPM2C is a protein similar to members of the protein phosphatase 2C (PP2C) family. It is a catalytic subunit of the mitochondrial matrix phosphatase and is involved in reactivation of the pyruvate dehydrogenase complex.
Function:
Catalyzes the dephosphorylation and concomitant reactivation of the alpha subunit of the E1 component of the pyruvate dehydrogenase complex (By similarity).
Subunit:
Heterodimer of a catalytic (PDP1) and a regulatory (PDPR) subunit (By similarity).
Subcellular Location:
Mitochondrion matrix (By similarity).
DISEASE:
Defects in PDP1 are the cause of pyruvate dehydrogenase phosphatase deficiency (PDP deficiency) [MIM:608782]. PDP deficiency results in lactic acidosis leading to neurological dysfunction.
Similarity:
Belongs to the PP2C family.
Database links:
UniProtKB/Swiss-Prot: Q9P0J1.3
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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