Recombinant Human [Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrial (PDK3), Truncated

Uniprot Id

Q15120

Target Species

Human

Target Name

PDK3

Target Full Name

[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrial

Target Function

Inhibits pyruvate dehydrogenase activity by phosphorylation of the E1 subunit PDHA1, and thereby regulates glucose metabolism and aerobic respiration. Can also phosphorylate PDHA2. Decreases glucose utilization and increases fat metabolism in response to prolonged fasting, and as adaptation to a high-fat diet. Plays a role in glucose homeostasis and in maintaining normal blood glucose levels in function of nutrient levels and under starvation. Plays a role in the generation of reactive oxygen species.

Target Involvement

Charcot-Marie-Tooth disease, X-linked dominant, 6 (CMTX6)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

PDK/BCKDK protein kinase family

Target Tissue Specificity

Expressed in heart, skeletal muscle, spinal cord, as well as fetal and adult brain.

Target Synonyms

[Pyruvate dehydrogenase [lipoamide] kinase isozyme 3 mitochondrial; [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3; CMTX6; GS1-358P8.4; mitochondrial; PDK3; PDK3_HUMAN; Pyruvate dehydrogenase kinase isoenzyme 3; Pyruvate dehydrogenase kinase isoform 3; Pyruvate dehydrogenase kinase isozyme 3; Pyruvate dehydrogenase lipoamide kinase isozyme 3 mitochondrial

Target Background

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle, and thus is one of the major enzymes responsible for the regulation of glucose metabolism. The enzymatic activity of PDH is regulated by a phosphorylation/dephosphorylation cycle, and phosphorylation results in inactivation of PDH. The protein encoded by this gene is one of the three pyruvate dehydrogenase kinases that inhibits the PDH complex by phosphorylation of the E1 alpha subunit. This gene is predominantly expressed in the heart and skeletal muscles. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.