Rabbit anti-Human PDP1 Polyclonal Antibody

Uniprot Id

Q9P0J1

Target Species

Human

Target Name

PDP1

Target Full Name

[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial

Target Function

Catalyzes the dephosphorylation and concomitant reactivation of the alpha subunit of the E1 component of the pyruvate dehydrogenase complex.

Target Involvement

Pyruvate dehydrogenase phosphatase deficiency (PDP deficiency)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

PP2C family

Target Synonyms

[Pyruvate dehydrogenase [acetyl transferring]] phosphatase 1; mitochondrial; [Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1; FLJ32517; Gm1024; MGC119646 ; mitochondrial; PDH; PDP 1; PDP; Pdp1; PDP1_HUMAN; PDPC 1; PDPC; PPM2C; Protein phosphatase 2C; protein phosphatase 2C; magnesium dependent; catalytic subunit; Pyruvate dehydrogenase (Lipoamide) phosphatase phosphatase; Pyruvate dehydrogenase phosphatase catalytic subunit 1; Pyruvate dehydrogenase phosphatase; catalytic subunit 1; RP23 203A12.1

Target Background

Pyruvate dehydrogenase (E1) is one of the three components (E1, E2, and E3) of the large pyruvate dehydrogenase complex. Pyruvate dehydrogenase kinases catalyze phosphorylation of serine residues of E1 to inactivate the E1 component and inhibit the complex. Pyruvate dehydrogenase phosphatases catalyze the dephosphorylation and activation of the E1 component to reverse the effects of pyruvate dehydrogenase kinases. Pyruvate dehydrogenase phosphatase is a heterodimer consisting of catalytic and regulatory subunits. Two catalytic subunits have been reported; one is predominantly expressed in skeletal muscle and another one is is much more abundant in the liver. The catalytic subunit, encoded by this gene, is the former, and belongs to the protein phosphatase 2C (PP2C) superfamily. Along with the pyruvate dehydrogenase complex and pyruvate dehydrogenase kinases, this enzyme is located in the mitochondrial matrix. Mutation in this gene causes pyruvate dehydrogenase phosphatase deficiency. Multiple alternatively spliced transcript variants encoding different isoforms have been identified.