Gene/Proteome Database (LMPD)

LMPD ID
LMP000528
Gene ID
5165
Species
Homo sapiens (Human)
Gene Name
pyruvate dehydrogenase kinase, isozyme 3
Gene Symbol
PDK3
Synonyms
CMTX6; GS1-358P8.4
Alternate Names
pyruvate dehydrogenase kinase, isozyme 3; pyruvate dehydrogenase kinase, isoenzyme 3; pyruvate dehydrogenase, lipoamide, kinase isozyme 3, mitochondrial; [Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrial
Chromosome
X
Map Location
Xp22.11
EC Number
2.7.11.2
Summary
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. [provided by RefSeq, Mar 2010]
Orthologs
View orthologs and multiple alignments for PDK3

Proteins

pyruvate dehydrogenase kinase, isozyme 3 isoform 1 precursor
Refseq ID NP_001135858
Protein GI 215422338
UniProt ID Q15120
mRNA ID NM_001142386
Length 415
RefSeq Status REVIEWED
MRLFRWLLKQPVPKQIERYSRFSPSPLSIKQFLDFGRDNACEKTSYMFLRKELPVRLANTMREVNLLPDNLLNRPSVGLVQSWYMQSFLELLEYENKSPEDPQVLDNFLQVLIKVRNRHNDVVPTMAQGVIEYKEKFGFDPFISTNIQYFLDRFYTNRISFRMLINQHTLLFGGDTNPVHPKHIGSIDPTCNVADVVKDAYETAKMLCEQYYLVAPELEVEEFNAKAPDKPIQVVYVPSHLFHMLFELFKNSMRATVELYEDRKEGYPAVKTLVTLGKEDLSIKISDLGGGVPLRKIDRLFNYMYSTAPRPSLEPTRAAPLAGFGYGLPISRLYARYFQGDLKLYSMEGVGTDAVIYLKALSSESFERLPVFNKSAWRHYKTTPEADDWSNPSSEPRDASKYKAKQDKIKTNRTF
pyruvate dehydrogenase kinase, isozyme 3 isoform 2 precursor
Refseq ID NP_005382
Protein GI 4885545
UniProt ID Q15120
mRNA ID NM_005391
Length 406
RefSeq Status REVIEWED
MRLFRWLLKQPVPKQIERYSRFSPSPLSIKQFLDFGRDNACEKTSYMFLRKELPVRLANTMREVNLLPDNLLNRPSVGLVQSWYMQSFLELLEYENKSPEDPQVLDNFLQVLIKVRNRHNDVVPTMAQGVIEYKEKFGFDPFISTNIQYFLDRFYTNRISFRMLINQHTLLFGGDTNPVHPKHIGSIDPTCNVADVVKDAYETAKMLCEQYYLVAPELEVEEFNAKAPDKPIQVVYVPSHLFHMLFELFKNSMRATVELYEDRKEGYPAVKTLVTLGKEDLSIKISDLGGGVPLRKIDRLFNYMYSTAPRPSLEPTRAAPLAGFGYGLPISRLYARYFQGDLKLYSMEGVGTDAVIYLKALSSESFERLPVFNKSAWRHYKTTPEADDWSNPSSEPRDASKYKAKQ

Gene Information

Entrez Gene ID
5165
Gene Name
pyruvate dehydrogenase kinase, isozyme 3
Gene Symbol
PDK3
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 TAS:ProtInc C mitochondrion
GO:0005524 IDA:UniProtKB F ATP binding
GO:0004672 IDA:UniProtKB F protein kinase activity
GO:0004674 IDA:UniProtKB F protein serine/threonine kinase activity
GO:0004740 IDA:UniProtKB F pyruvate dehydrogenase (acetyl-transferring) kinase activity
GO:0008219 IEA:UniProtKB-KW P cell death
GO:0044237 TAS:Reactome P cellular metabolic process
GO:0071398 IMP:UniProtKB P cellular response to fatty acid
GO:0071333 ISS:UniProtKB P cellular response to glucose stimulus
GO:0006006 IEA:UniProtKB-KW P glucose metabolic process
GO:0097411 IMP:UniProtKB P hypoxia-inducible factor-1alpha signaling pathway
GO:0018105 IDA:UniProtKB P peptidyl-serine phosphorylation
GO:0035357 IMP:UniProtKB P peroxisome proliferator activated receptor signaling pathway
GO:0006090 TAS:Reactome P pyruvate metabolic process
GO:0010510 IMP:UniProtKB P regulation of acetyl-CoA biosynthetic process from pyruvate
GO:0010906 IMP:UniProtKB P regulation of glucose metabolic process
GO:2000377 IMP:UniProtKB P regulation of reactive oxygen species metabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_2071 Pyruvate metabolism
REACT_1046 Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_12528 Regulation of pyruvate dehydrogenase (PDH) complex
REACT_111083 The citric acid (TCA) cycle and respiratory electron transport

Domain Information

InterPro Annotations

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

UniProt Annotations

Entry Information

Gene Name
pyruvate dehydrogenase kinase, isozyme 3
Protein Entry
PDK3_HUMAN
UniProt ID
Q15120
Species
Human

Comments

Comment Type Description
Alternative Products Event=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 Activity ATP + [pyruvate dehydrogenase (acetyl- transferring)] = ADP + [pyruvate dehydrogenase (acetyl- transferring)] phosphate. {ECO
Disease Charcot-Marie-Tooth disease, X-linked dominant, 6 (CMTX6) [MIM
Enzyme Regulation Activated by interaction with DLAT. Inhibited by AZD7545, dichloroacetate and radicicol.
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
Induction Up-regulated in response to hypoxia. Up-regulated in response to fatty acids. Up-regulated by PPARD. {ECO
Similarity Belongs to the PDK/BCKDK protein kinase family.
Similarity Contains 1 histidine kinase domain.
Subcellular Location Mitochondrion matrix.
Subunit Homodimer. 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 Specificity Expressed in heart, skeletal muscle, spinal cord, as well as fetal and adult brain.

Identical and Related Proteins

Unique RefSeq proteins for LMP000528 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
215422338 RefSeq NP_001135858 415 pyruvate dehydrogenase kinase, isozyme 3 isoform 1 precursor
4885545 RefSeq NP_005382 406 pyruvate dehydrogenase kinase, isozyme 3 isoform 2 precursor

Identical Sequences to LMP000528 proteins

Reference Database Accession Length Protein Name
GI:4885545 GenBank AAH15948.1 406 Pyruvate dehydrogenase kinase, isozyme 3 [Homo sapiens]
GI:4885545 GenBank EAW99020.1 406 pyruvate dehydrogenase kinase, isozyme 3, isoform CRA_b [Homo sapiens]
GI:215422338 GenBank JAA36538.1 415 pyruvate dehydrogenase kinase, isozyme 3 [Pan troglodytes]
GI:4885545 GenBank AHD71033.1 406 Sequence 4501 from patent US 8586006
GI:4885545 GenBank AIC49360.1 406 PDK3, partial [synthetic construct]
GI:4885545 GenBank AIC62544.1 406 PDK3, partial [synthetic construct]
GI:215422338 RefSeq XP_003819631.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Pan paniscus]
GI:215422338 RefSeq XP_003917562.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Papio anubis]
GI:215422338 RefSeq XP_005593248.1 415 PREDICTED: [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3, mitochondrial [Macaca fascicularis]
GI:215422338 RefSeq XP_007989505.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 isoform X1 [Chlorocebus sabaeus]
GI:215422338 RefSeq XP_010351525.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Rhinopithecus roxellana]
GI:4885545 SwissProt Q15120.1 406 RecName: Full=[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrial; AltName: Full=Pyruvate dehydrogenase kinase isoform 3; Flags: Precursor [Homo sapiens]

Related Sequences to LMP000528 proteins

Reference Database Accession Length Protein Name
GI:4885545 DBBJ BAG63378.1 415 unnamed protein product [Homo sapiens]
GI:4885545 GenBank AAQ02417.1 407 pyruvate dehydrogenase kinase, isoenzyme 3, partial [synthetic construct]
GI:215422338 RefSeq XP_537984.2 415 PREDICTED: [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3, mitochondrial isoformX1 [Canis lupus familiaris]
GI:215422338 RefSeq XP_001927474.1 415 PREDICTED: [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3, mitochondrial isoform 1 [Sus scrofa]
GI:215422338 RefSeq XP_002720035.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Oryctolagus cuniculus]
GI:4885545 RefSeq XP_002831515.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Pongo abelii]
GI:215422338 RefSeq XP_003261208.1 415 PREDICTED: [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3, mitochondrial isoform 1 [Nomascus leucogenys]
GI:4885545 RefSeq XP_003819631.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Pan paniscus]
GI:4885545 RefSeq XP_007989505.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 isoform X1 [Chlorocebus sabaeus]
GI:215422338 RefSeq XP_008064012.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 isoform X1 [Tarsius syrichta]
GI:215422338 RefSeq XP_003317444.3 415 PREDICTED: LOW QUALITY PROTEIN: pyruvate dehydrogenase kinase, isozyme 3 [Pan troglodytes]
GI:4885545 RefSeq XP_010351525.1 415 PREDICTED: pyruvate dehydrogenase kinase, isozyme 3 [Rhinopithecus roxellana]