InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PDK3

Summary

InnateDB Protein

IDBP-52917.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PDK3

Protein Name

pyruvate dehydrogenase kinase, isozyme 3

Synonyms

CMTX6; GS1-358P8.4;

Species

Homo sapiens

Ensembl Protein

ENSP00000368460

InnateDB Gene

IDBG-52915 (PDK3)

Protein Structure

UniProt Annotation

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. {ECO:0000269PubMed:10748134, ECO:0000269PubMed:11486000, ECO:0000269PubMed:15861126, ECO:0000269PubMed:16436377, ECO:0000269PubMed:17683942, ECO:0000269PubMed:18718909, ECO:0000269PubMed:22865452}.

Subcellular Localization

Mitochondrion matrix.

Disease Associations

Charcot-Marie-Tooth disease, X-linked dominant, 6 (CMTX6) [MIM:300905]: A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy. {ECO:0000269PubMed:23297365}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Expressed in heart, skeletal muscle, spinal cord, as well as fetal and adult brain. {ECO:0000269PubMed:23297365}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 10 experimentally validated interaction(s) in this database.

Experimentally validated
Total	10 [view]
Protein-Protein	8 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	2 [view]
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004672	protein kinase activity
GO:0004674	protein serine/threonine kinase activity
GO:0004740	pyruvate dehydrogenase (acetyl-transferring) kinase activity
GO:0005515	protein binding
GO:0005524	ATP binding

Biological Process

GO:0006006	glucose metabolic process
GO:0006090	pyruvate metabolic process
GO:0008219	cell death
GO:0010510	regulation of acetyl-CoA biosynthetic process from pyruvate
GO:0010906	regulation of glucose metabolic process
GO:0018105	peptidyl-serine phosphorylation
GO:0035357	peroxisome proliferator activated receptor signaling pathway
GO:0044237	cellular metabolic process
GO:0044281	small molecule metabolic process
GO:0071333	cellular response to glucose stimulus
GO:0071398	cellular response to fatty acid
GO:0097411	hypoxia-inducible factor-1alpha signaling pathway
GO:2000377	regulation of reactive oxygen species metabolic process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix

Protein Structure and Domains

PDB ID

InterPro

IPR003594 Histidine kinase-like ATPase, C-terminal domain
IPR005467 Signal transduction histidine kinase, core
IPR018955 Branched-chain alpha-ketoacid dehydrogenase kinase/Pyruvate dehydrogenase kinase, N-terminal

PFAM

PF02518
PF13581
PF10436

PRINTS

PIRSF

SMART

SM00387

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q15120