LMPD Database

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LMP000528

UniProt Annotations

Entry Information
Gene Namepyruvate dehydrogenase kinase, isozyme 3
Protein EntryPDK3_HUMAN
UniProt IDQ15120
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q15120-1; Sequence=Displayed; Name=2; IsoId=Q15120-2; Sequence=VSP_043365; Note=No experimental confirmation available.;
Catalytic ActivityATP + [pyruvate dehydrogenase (acetyl- transferring)] = ADP + [pyruvate dehydrogenase (acetyl- transferring)] phosphate. {ECO
DiseaseCharcot-Marie-Tooth disease, X-linked dominant, 6 (CMTX6) [MIM
Enzyme RegulationActivated by interaction with DLAT. Inhibited by AZD7545, dichloroacetate and radicicol.
FunctionInhibits 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. {ECO
InductionUp-regulated in response to hypoxia. Up-regulated in response to fatty acids. Up-regulated by PPARD. {ECO
SimilarityBelongs to the PDK/BCKDK protein kinase family.
SimilarityContains 1 histidine kinase domain.
Subcellular LocationMitochondrion matrix.
SubunitHomodimer. Interacts with the pyruvate dehydrogenase complex subunit DLAT, and is part of the multimeric pyruvate dehydrogenase complex that contains multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). {ECO
Tissue SpecificityExpressed in heart, skeletal muscle, spinal cord, as well as fetal and adult brain.