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Details on Person UniProt:P00558 PGK1

Class:IdReferenceGeneProduct:61610
_chainChangeLoginitiator methionine:1 added on Fri February 6 2015;chain:2-417 added on Fri February 6 2015;initiator methionine:1 for 61610 removed on Fri Nov 03 2023;initiator methionine: for 61610 added on Fri Nov 03 2023;initiator methionine: for 61610 removed on Fri Aug 15 2025;initiator methionine:1 for 61610 added on Fri Aug 15 2025
_displayNameUniProt:P00558 PGK1
_timestamp2025-08-15 21:02:09
chaininitiator methionine:1
chain:2-417
checksumB5DFC7B5FA01767C
commentFUNCTION Catalyzes one of the two ATP producing reactions in the glycolytic pathway via the reversible conversion of 1,3-diphosphoglycerate to 3-phosphoglycerate (PubMed:30323285, PubMed:7391028). Both L- and D- forms of purine and pyrimidine nucleotides can be used as substrates, but the activity is much lower on pyrimidines (PubMed:18463139). In addition to its role as a glycolytic enzyme, it seems that PGK1 acts as a polymerase alpha cofactor protein (primer recognition protein) (PubMed:2324090). Acts as a protein kinase when localized to the mitochondrion where it phosphorylates pyruvate dehydrogenase kinase PDK1 to inhibit pyruvate dehydrogenase complex activity and suppress the formation of acetyl-coenzyme A from pyruvate, and consequently inhibit oxidative phosphorylation and promote glycolysis (PubMed:26942675, PubMed:36849569). May play a role in sperm motility (PubMed:26677959).CATALYTIC ACTIVITY (2R)-3-phosphoglycerate + ATP = (2R)-3-phospho-glyceroyl phosphate + ADPCATALYTIC ACTIVITY L-seryl-[protein] + ATP = O-phospho-L-seryl-[protein] + ADP + H(+)COFACTOR Specifically inhibited by heterocyclic compound CBR-470-0.BIOPHYSICOCHEMICAL PROPERTIES Carbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 2/5.SUBUNIT Monomer (PubMed:18463139). Interacts with kinase MAPK1/ERK2; the interaction is direct, occurs under hypoxic conditions, and promotes its interaction with PIN1 (PubMed:26942675). Interacts with peptidyl-prolyl cis-trans isomerase PIN1; the interaction is direct, occurs under hypoxic conditions, and targets the protein to the mitochondrion by promoting interactions with the TOM complex (PubMed:26942675). Interacts with mitochondrial circRNA mcPGK1 (via its 2nd stem-loop); the interaction is direct and targets the protein to the mitochondrion by promoting interactions with the TOM complex (PubMed:36849569). Interacts with pyruvate dehydrogenase kinase PDK1; the interaction is direct, occurs under hypoxic conditions and leads to PDK1-mediated inhibition of pyruvate dehydrogenase complex activity (PubMed:26942675).INTERACTION Hypoxic conditions promote mitochondrial targeting (PubMed:26942675). Targeted to the mitochondrion following phosphorylation by MAPK1/ERK2, cis-trans isomerization by PIN1, and binding to mitochondrial circRNA mcPGK1 (PubMed:36849569).ALTERNATIVE PRODUCTS Mainly expressed in spermatogonia. Localized on the principle piece in the sperm (at protein level). Expression significantly decreased in the testis of elderly men.PTM Phosphorylated at Ser-203 by MAPK1/ERK2 under hypoxic conditions, which promotes its mitochondrial targeting.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS Dysregulated mitochondrial targeting of PGK1 may contribute to the elevation of aerobic glycolysis seen in tumor cells, a phenomenon known as the Warburg effect.SIMILARITY Belongs to the phosphoglycerate kinase family.ONLINE INFORMATION Phosphoglycerate kinase entry
descriptionrecommendedName: Phosphoglycerate kinase 1 ecNumber evidence="10"2.7.11.1 ecNumber evidence="13"2.7.2.3 alternativeName: Cell migration-inducing gene 10 protein alternativeName: Primer recognition protein 2 shortName: PRP 2
geneNamePGK1
PGKA
MIG10
OK/SW-cl.110
identifierP00558
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
ATP-binding
Cytoplasm
Direct protein sequencing
Disease variant
Glycolysis
Hereditary hemolytic anemia
Hydroxylation
Kinase
Magnesium
Metal-binding
Mitochondrion
Nucleotide-binding
Phosphoprotein
Proteomics identification
Reference proteome
Transferase
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
namePGK1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8957921] ENSEMBL:ENSG00000102144 PGK1 [Homo sapiens]
secondaryIdentifierPGK1_HUMAN
A8K4W6
B7Z7A9
Q5J7W1
Q6IBT6
Q8NI87
sequenceLength417
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8967545] UniProt:P00558-1 PGK1 [Homo sapiens]
[ReferenceIsoform:8967546] UniProt:P00558-2 PGK1 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:70483] PGK1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:8958573] PGK1 [extracellular region] [Homo sapiens]
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