Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase 3

Gene

PFKFB3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Synthesis and degradation of fructose 2,6-bisphosphate.

Catalytic activityi

Beta-D-fructose 2,6-bisphosphate + H2O = D-fructose 6-phosphate + phosphate.
ATP + D-fructose 6-phosphate = ADP + beta-D-fructose 2,6-bisphosphate.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei75Fructose 6-phosphate1 Publication1
Binding sitei99Fructose 6-phosphate1 Publication1
Active sitei125Sequence analysis1
Binding sitei127Fructose 6-phosphate1 Publication1
Binding sitei133Fructose 6-phosphate1 Publication1
Active sitei155Sequence analysis1
Binding sitei169Fructose 6-phosphate1 Publication1
Binding sitei190Fructose 6-phosphate1 Publication1
Binding sitei194Fructose 6-phosphate1 Publication1
Binding sitei253Fructose 2,6-bisphosphate1 Publication1
Sitei253Transition state stabilizer1 Publication1
Active sitei254Tele-phosphohistidine intermediate1 Publication1
Binding sitei260Fructose 2,6-bisphosphate1 Publication1
Sitei260Transition state stabilizer1 Publication1
Binding sitei266Fructose 2,6-bisphosphate; via amide nitrogen1 Publication1
Active sitei323Proton donor/acceptor1 Publication1
Binding sitei334Fructose 2,6-bisphosphate1 Publication1
Binding sitei348Fructose 2,6-bisphosphate1 Publication1
Binding sitei352Fructose 2,6-bisphosphate1 Publication1
Binding sitei363Fructose 2,6-bisphosphate1 Publication1
Sitei388Transition state stabilizer1 Publication1
Binding sitei389Fructose 2,6-bisphosphate1 Publication1
Binding sitei393Fructose 2,6-bisphosphateBy similarity1
Binding sitei425ATP3 Publications1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi42 – 50ATP3 Publications9
Nucleotide bindingi164 – 169ATP3 Publications6
Nucleotide bindingi345 – 348ATPBy similarity4
Nucleotide bindingi389 – 393ATPBy similarity5

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionHydrolase, Kinase, Multifunctional enzyme, Transferase
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS10144-MONOMER
BRENDAi2.7.1.105 2681
3.1.3.46 2681
ReactomeiR-HSA-70171 Glycolysis
SABIO-RKiQ16875
SIGNORiQ16875

Names & Taxonomyi

Protein namesi
Recommended name:
6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase 3
Short name:
6PF-2-K/Fru-2,6-P2ase 3
Short name:
PFK/FBPase 3
Alternative name(s):
6PF-2-K/Fru-2,6-P2ase brain/placenta-type isozyme
Renal carcinoma antigen NY-REN-56
iPFK-2
Including the following 2 domains:
Fructose-2,6-bisphosphatase (EC:3.1.3.46)
Gene namesi
Name:PFKFB3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

EuPathDBiHostDB:ENSG00000170525.18
HGNCiHGNC:8874 PFKFB3
MIMi605319 gene
neXtProtiNX_Q16875

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Pathology & Biotechi

Organism-specific databases

DisGeNETi5209
OpenTargetsiENSG00000170525
PharmGKBiPA33213

Chemistry databases

ChEMBLiCHEMBL2331053
GuidetoPHARMACOLOGYi2937

Polymorphism and mutation databases

BioMutaiPFKFB3
DMDMi3023733

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001799681 – 5206-phosphofructo-2-kinase/fructose-2,6-bisphosphatase 3Add BLAST520

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei461Phosphoserine; by AMPKCombined sources1 Publication1
Modified residuei463PhosphothreonineCombined sources1
Modified residuei467PhosphoserineCombined sources1
Modified residuei471Phosphothreonine; by PKCBy similarity1

Post-translational modificationi

Phosphorylation by AMPK stimulates activity.1 Publication

Keywords - PTMi

Phosphoprotein

Proteomic databases

PaxDbiQ16875
PeptideAtlasiQ16875
PRIDEiQ16875

PTM databases

DEPODiQ16875
iPTMnetiQ16875
PhosphoSitePlusiQ16875

Expressioni

Tissue specificityi

Ubiquitous.

Gene expression databases

BgeeiENSG00000170525
CleanExiHS_PFKFB3
ExpressionAtlasiQ16875 baseline and differential
GenevisibleiQ16875 HS

Organism-specific databases

HPAiCAB020795
HPA008266
HPA043889

Interactioni

Protein-protein interaction databases

BioGridi111230, 15 interactors
DIPiDIP-33964N
IntActiQ16875, 7 interactors
STRINGi9606.ENSP00000369100

Chemistry databases

BindingDBiQ16875

Structurei

Secondary structure

1520
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi5 – 7Combined sources3
Turni9 – 11Combined sources3
Beta strandi14 – 16Combined sources3
Beta strandi36 – 43Combined sources8
Beta strandi44 – 47Combined sources4
Helixi48 – 61Combined sources14
Beta strandi66 – 70Combined sources5
Helixi71 – 78Combined sources8
Beta strandi79 – 81Combined sources3
Helixi85 – 88Combined sources4
Helixi93 – 116Combined sources24
Beta strandi120 – 126Combined sources7
Helixi131 – 144Combined sources14
Beta strandi147 – 154Combined sources8
Helixi157 – 163Combined sources7
Helixi165 – 169Combined sources5
Helixi173 – 175Combined sources3
Helixi180 – 195Combined sources16
Turni203 – 209Combined sources7
Beta strandi210 – 216Combined sources7
Turni217 – 220Combined sources4
Beta strandi221 – 225Combined sources5
Helixi230 – 241Combined sources12
Beta strandi249 – 253Combined sources5
Helixi258 – 261Combined sources4
Helixi273 – 289Combined sources17
Beta strandi295 – 298Combined sources4
Helixi302 – 309Combined sources8
Turni310 – 312Combined sources3
Beta strandi315 – 317Combined sources3
Helixi319 – 321Combined sources3
Helixi327 – 329Combined sources3
Helixi334 – 340Combined sources7
Helixi342 – 350Combined sources9
Turni352 – 354Combined sources3
Helixi363 – 379Combined sources17
Beta strandi381 – 387Combined sources7
Helixi389 – 399Combined sources11
Turni404 – 406Combined sources3
Helixi407 – 409Combined sources3
Beta strandi416 – 423Combined sources8
Beta strandi426 – 433Combined sources8
Helixi442 – 446Combined sources5
Helixi455 – 457Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2AXNX-ray2.10A1-520[»]
2DWOX-ray2.25A1-520[»]
2DWPX-ray2.70A1-520[»]
2I1VX-ray2.50B1-520[»]
3QPUX-ray2.30A1-520[»]
3QPVX-ray2.50A1-520[»]
3QPWX-ray2.25A1-520[»]
4D4JX-ray3.00A1-449[»]
4D4KX-ray3.24A1-449[»]
4D4LX-ray3.16A1-449[»]
4D4MX-ray2.32A1-449[»]
4MA4X-ray2.23A1-520[»]
5AJVX-ray3.01B1-520[»]
5AJWX-ray2.50A1-520[»]
5AJXX-ray2.58A1-520[»]
5AJYX-ray2.37A1-520[»]
5AJZX-ray2.35A1-520[»]
5AK0X-ray2.03A1-520[»]
ProteinModelPortaliQ16875
SMRiQ16875
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ16875

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni1 – 2456-phosphofructo-2-kinaseAdd BLAST245
Regioni246 – 520Fructose-2,6-bisphosphataseAdd BLAST275

Sequence similaritiesi

In the C-terminal section; belongs to the phosphoglycerate mutase family.Curated

Phylogenomic databases

eggNOGiKOG0234 Eukaryota
COG0406 LUCA
GeneTreeiENSGT00390000018751
HOGENOMiHOG000181112
HOVERGENiHBG005628
InParanoidiQ16875
KOiK01103
OMAiMKRINCY
OrthoDBiEOG091G0A43
PhylomeDBiQ16875
TreeFamiTF313541

Family and domain databases

CDDicd07067 HP_PGM_like, 1 hit
Gene3Di3.40.50.1240, 1 hit
InterProiView protein in InterPro
IPR003094 6Pfruct_kin
IPR013079 6Phosfructo_kin
IPR013078 His_Pase_superF_clade-1
IPR029033 His_PPase_superfam
IPR027417 P-loop_NTPase
IPR001345 PG/BPGM_mutase_AS
PANTHERiPTHR10606 PTHR10606, 1 hit
PfamiView protein in Pfam
PF01591 6PF2K, 1 hit
PF00300 His_Phos_1, 1 hit
PRINTSiPR00991 6PFRUCTKNASE
SMARTiView protein in SMART
SM00855 PGAM, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
SSF53254 SSF53254, 1 hit
PROSITEiView protein in PROSITE
PS00175 PG_MUTASE, 1 hit

Sequences (4)i

Sequence statusi: Complete.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q16875-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MPLELTQSRV QKIWVPVDHR PSLPRSCGPK LTNSPTVIVM VGLPARGKTY
60 70 80 90 100
ISKKLTRYLN WIGVPTKVFN VGEYRREAVK QYSSYNFFRP DNEEAMKVRK
110 120 130 140 150
QCALAALRDV KSYLAKEGGQ IAVFDATNTT RERRHMILHF AKENDFKAFF
160 170 180 190 200
IESVCDDPTV VASNIMEVKI SSPDYKDCNS AEAMDDFMKR ISCYEASYQP
210 220 230 240 250
LDPDKCDRDL SLIKVIDVGR RFLVNRVQDH IQSRIVYYLM NIHVQPRTIY
260 270 280 290 300
LCRHGENEHN LQGRIGGDSG LSSRGKKFAS ALSKFVEEQN LKDLRVWTSQ
310 320 330 340 350
LKSTIQTAEA LRLPYEQWKA LNEIDAGVCE ELTYEEIRDT YPEEYALREQ
360 370 380 390 400
DKYYYRYPTG ESYQDLVQRL EPVIMELERQ ENVLVICHQA VLRCLLAYFL
410 420 430 440 450
DKSAEEMPYL KCPLHTVLKL TPVAYGCRVE SIYLNVESVC THRERSEDAK
460 470 480 490 500
KGPNPLMRRN SVTPLASPEP TKKPRINSFE EHVASTSAAL PSCLPPEVPT
510 520
QLPGQNMKGS RSSADSSRKH
Length:520
Mass (Da):59,609
Last modified:November 1, 1996 - v1
Checksum:iA7675A4ADC376879
GO
Isoform 2 (identifier: Q16875-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     506-520: NMKGSRSSADSSRKH → PLLGQACLT

Show »
Length:514
Mass (Da):58,876
Checksum:i18FD1D267306853D
GO
Isoform 3 (identifier: Q16875-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-26: MPLELTQSRVQKIWVPVDHRPSLPRS → MPFRKA

Note: Gene prediction based on EST data.
Show »
Length:500
Mass (Da):57,287
Checksum:i32D2924D3D207C17
GO
Isoform 4 (identifier: Q16875-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-26: MPLELTQSRVQKIWVPVDHRPSLPRS → MGEGGQKEGDSQQAGALPLLCQLDTFSPKATVFGVSINPA

Note: No experimental confirmation available.
Show »
Length:534
Mass (Da):60,602
Checksum:iFB3222408A8BC7EE
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti136M → V in AAB99795 (Ref. 4) Curated1
Sequence conflicti141A → G in AAC62000 (PubMed:10077634).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0471651 – 26MPLEL…SLPRS → MPFRKA in isoform 3. CuratedAdd BLAST26
Alternative sequenceiVSP_0545491 – 26MPLEL…SLPRS → MGEGGQKEGDSQQAGALPLL CQLDTFSPKATVFGVSINPA in isoform 4. 1 PublicationAdd BLAST26
Alternative sequenceiVSP_004680506 – 520NMKGS…SSRKH → PLLGQACLT in isoform 2. 1 PublicationAdd BLAST15

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D49817 mRNA Translation: BAA08624.1
L77662 mRNA Translation: AAL40083.1
AF109735 mRNA Translation: AAD08818.1
AF041831
, AF041823, AF041824, AF041825, AF041826, AF041827, AF041828, AF041829, AF041830 Genomic DNA Translation: AAB99795.1
AF056320 mRNA Translation: AAC62000.1
AK291263 mRNA Translation: BAF83952.1
AK304450 mRNA Translation: BAH14191.1
AL359960 Genomic DNA No translation available.
AL157395 Genomic DNA No translation available.
CH471072 Genomic DNA Translation: EAW86398.1
CH471072 Genomic DNA Translation: EAW86399.1
BC040482 mRNA Translation: AAH40482.1
CCDSiCCDS44353.1 [Q16875-3]
CCDS60479.1 [Q16875-4]
CCDS7078.1 [Q16875-1]
CCDS81439.1 [Q16875-2]
PIRiJC4626
RefSeqiNP_001269559.1, NM_001282630.2 [Q16875-4]
NP_001300992.1, NM_001314063.1 [Q16875-2]
NP_004557.1, NM_004566.3 [Q16875-1]
UniGeneiHs.195471

Genome annotation databases

EnsembliENST00000360521; ENSP00000353712; ENSG00000170525 [Q16875-2]
ENST00000379775; ENSP00000369100; ENSG00000170525 [Q16875-1]
ENST00000379789; ENSP00000369115; ENSG00000170525 [Q16875-3]
ENST00000536985; ENSP00000443319; ENSG00000170525 [Q16875-4]
GeneIDi5209
KEGGihsa:5209
UCSCiuc001ijd.4 human [Q16875-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiF263_HUMAN
AccessioniPrimary (citable) accession number: Q16875
Secondary accession number(s): B7Z955
, O43622, O75902, Q5VX15, Q5VX18, Q5VX19
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: November 1, 1996
Last modified: March 28, 2018
This is version 185 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health