PFKFB1

PFKFB1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1K6M
Identifiers
Aliases	PFKFB1 , F6PK, HL2K, PFRX, 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1
External IDs	OMIM: 311790 MGI: 107816 HomoloGene: 105654 GeneCards: PFKFB1
Gene location (Human)
Chr.	X chromosome (human)
End	54,998,534 bp
Gene location (Mouse)
Chr.	X chromosome (mouse)
End	149,426,874 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; gastrocnemius muscle; ; vastus lateralis muscle; ; biceps brachii; ; triceps brachii muscle; ; deltoid muscle; ; adipose tissue; ; subcutaneous adipose tissue; ; abdominal fat; ; tibialis anterior muscle;
	Top expressed in
	sternocleidomastoid muscle; ; vastus lateralis muscle; ; triceps brachii muscle; ; masseter muscle; ; tibialis anterior muscle; ; temporal muscle; ; digastric muscle; ; gastrocnemius muscle; ; right ventricle; ; plantaris muscle;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	transferase activity ; nucleotide binding ; fructose-6-phosphate binding ; kinase activity ; catalytic activity ; protein binding ; identical protein binding ; fructose-2,6-bisphosphate 2-phosphatase activity ; hydrolase activity ; ATP binding ; 6-phosphofructo-2-kinase activity ; kinase binding ;
Cellular component	cytosol ; 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase complex ;
Biological process	gluconeogenesis ; response to glucagon ; glycolytic process ; phosphorylation ; fructose metabolic process ; response to glucocorticoid ; carbohydrate phosphorylation ; response to insulin ; positive regulation of glucokinase activity ; fructose 2,6-bisphosphate metabolic process ; animal organ regeneration ; response to starvation ; metabolism ; response to cAMP ; dephosphorylation ; negative regulation of glycolytic process ; positive regulation of glycolytic process ;
	Sources:Amigo / QuickGO
Orthologs
	5207
	18639
	ENSG00000158571
	ENSMUSG00000025271
	P16118
	P70266
	NM_001271804 ; NM_001271805 ; NM_002625
NM_008824 ; NM_001374170 ; NM_001374171 ; NM_001374172 ; NM_001374173 ;
	NM_001374175
	NP_001258733 ; NP_001258734 ; NP_002616
NP_032850 ; NP_001361099 ; NP_001361100 ; NP_001361101 ; NP_001361102 ;
	NP_001361104
	Wikidata
View/Edit Human	View/Edit Mouse

Last updated September 05, 2022

6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1 is an enzyme that in humans is encoded by the PFKFB1 gene.^[5]^[6]

This gene encodes a member of the family of bifunctional 6-phosphofructo-2-kinase:fructose-2,6-biphosphatase enzymes. The enzyme forms a homodimer that catalyzes both the synthesis and degradation of fructose-2,6-biphosphate using independent catalytic domains. Fructose-2,6-biphosphate is an activator of the glycolysis pathway and an inhibitor of the gluconeogenesis pathway. Consequently, regulating fructose-2,6-biphosphate levels through the activity of this enzyme is thought to regulate glucose homeostasis.^[6]

Related Research Articles

Phosphofructokinase-2 (6-phosphofructo-2-kinase, PFK-2) or fructose bisphosphatase-2 (FBPase-2), is an enzyme indirectly responsible for regulating the rates of glycolysis and gluconeogenesis in cells. It catalyzes formation and degradation of a significant allosteric regulator, fructose-2,6-bisphosphate (Fru-2,6-P₂) from substrate fructose-6-phosphate. Fru-2,6-P₂ contributes to the rate-determining step of glycolysis as it activates enzyme phosphofructokinase 1 in the glycolysis pathway, and inhibits fructose-1,6-bisphosphatase 1 in gluconeogenesis. Since Fru-2,6-P₂ differentially regulates glycolysis and gluconeogenesis, it can act as a key signal to switch between the opposing pathways. Because PFK-2 produces Fru-2,6-P₂ in response to hormonal signaling, metabolism can be more sensitively and efficiently controlled to align with the organism's glycolytic needs. This enzyme participates in fructose and mannose metabolism. The enzyme is important in the regulation of hepatic carbohydrate metabolism and is found in greatest quantities in the liver, kidney and heart. In mammals, several genes often encode different isoforms, each of which differs in its tissue distribution and enzymatic activity. The family described here bears a resemblance to the ATP-driven phospho-fructokinases, however, they share little sequence similarity, although a few residues seem key to their interaction with fructose 6-phosphate.

Fructose 2,6-bisphosphate, abbreviated Fru-2,6-P₂, is a metabolite that allosterically affects the activity of the enzymes phosphofructokinase 1 (PFK-1) and fructose 1,6-bisphosphatase (FBPase-1) to regulate glycolysis and gluconeogenesis. Fru-2,6-P₂ itself is synthesized and broken down by the bifunctional enzyme phosphofructokinase 2/fructose-2,6-bisphosphatase (PFK-2/FBPase-2).

Glucosamine—fructose-6-phosphate aminotransferase isomerizing 1 is an enzyme that in humans is encoded by the GFPT1 gene.

PFKFB3 is a gene that encodes the 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 3 enzyme in humans. It is one of 4 tissue-specific PFKFB isoenzymes identified currently (PFKFB1-4).

Tribbles homolog 3 is a protein that in humans is encoded by the TRIB3 gene.

Dimethylaniline monooxygenase [N-oxide-forming] 1 is an enzyme that in humans is encoded by the FMO1 gene.

Phosphofructokinase, platelet, also known as PFKP is an enzyme which in humans is encoded by the PFKP gene.

Serine/threonine protein kinase NLK is an enzyme that in humans is encoded by the NLK gene. Its name is an abbreviation for Nemo-Like Kinase, Nemo (nmo) being the Drosophila ortholog of the mammalian NLK gene. This enzyme is a member of the Mitogen-activated protein kinase (MAPK) family, although not explicitly designated as such. It is a highly divergent, atypical member of the MAPK group, lacking most features so characteristic of most mitogen-activated protein kinases. Its activation mechanism and downstream targets are still not well characterized.

6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 2 is an enzyme that in humans is encoded by the PFKFB2 gene.

Bile acid-CoA:amino acid N-acyltransferase is an enzyme that in humans is encoded by the BAAT gene.

Phosphorylase b kinase regulatory subunit beta is an enzyme that in humans is encoded by the PHKB gene.

Phosphorylase b kinase gamma catalytic chain, testis/liver isoform is an enzyme that in humans is encoded by the PHKG2 gene.

Serine/threonine-protein kinase 10 is an enzyme that in humans is encoded by the STK10 gene.

Thymidylate kinase also known as deoxythymidylate kinase or dTMP kinase is an enzyme that in humans is encoded by the DTYMK gene. and belongs to thymidylate kinase family of proteins.

Inositol hexakisphosphate kinase 1 is an enzyme that in humans is encoded by the IP6K1 gene.

Mitogen-activated protein kinase kinase kinase 9 is an enzyme that in humans is encoded by the MAP3K9 gene.

CTP synthase 2 is an enzyme that in humans is encoded by the CTPS2 gene.

Cyclin-dependent kinase-like 2 is an enzyme that in humans is encoded by the CDKL2 gene.

6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 4 also known as PFKFB4 is an enzyme which in humans is encoded by the PFKFB4 gene.

Fructose-1,6-bisphosphatase 1 is a protein that in humans is encoded by the FBP1 gene.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000158571 - Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000025271 - Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ Batra RS, Hatchwell E, Rider S, Brown R, Brown GK, Craig IW (Apr 1997). "Localization of human liver 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase (PFKFB1) within a YAC contig in Xp11.21". Genomics. 40 (2): 358–61. doi:10.1006/geno.1996.4530. PMID 9119406.
1 2 "Entrez Gene: PFKFB1 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1".

Lange AJ, Pilkis SJ (1990). "Sequence of human liver 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase". Nucleic Acids Res. 18 (12): 3652. doi:10.1093/nar/18.12.3652. PMC 331036 . PMID 2163524.
Marker AJ, Colosia AD, Tauler A, et al. (1989). "Glucocorticoid regulation of hepatic 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase gene expression". J. Biol. Chem. 264 (12): 7000–4. doi: 10.1016/S0021-9258(18)83531-1 . PMID 2540168.
Algaier J, Uyeda K (1988). "Molecular cloning, sequence analysis, and expression of a human liver cDNA coding for fructose-6-P,2-kinase:fructose-2,6-bisphosphatase". Biochem. Biophys. Res. Commun. 153 (1): 328–33. doi:10.1016/S0006-291X(88)81226-9. PMID 2837207.
Lee YH, Li Y, Uyeda K, Hasemann CA (2003). "Tissue-specific structure/function differentiation of the liver isoform of 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase". J. Biol. Chem. 278 (1): 523–30. doi: 10.1074/jbc.M209105200 . PMID 12379646.
Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. Bibcode:2002PNAS...9916899M. doi: 10.1073/pnas.242603899 . PMC 139241 . PMID 12477932.
Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928 . PMID 15489334.
Ross MT, Grafham DV, Coffey AJ, et al. (2005). "The DNA sequence of the human X chromosome". Nature. 434 (7031): 325–37. Bibcode:2005Natur.434..325R. doi:10.1038/nature03440. PMC 2665286 . PMID 15772651.
Kimura K, Wakamatsu A, Suzuki Y, et al. (2006). "Diversification of transcriptional modulation: Large-scale identification and characterization of putative alternative promoters of human genes". Genome Res. 16 (1): 55–65. doi:10.1101/gr.4039406. PMC 1356129 . PMID 16344560.

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[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000158571 - Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000025271 - Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid9119406-5] Batra RS, Hatchwell E, Rider S, Brown R, Brown GK, Craig IW (Apr 1997). "Localization of human liver 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase (PFKFB1) within a YAC contig in Xp11.21". Genomics. 40 (2): 358–61. doi:10.1006/geno.1996.4530. PMID 9119406.

[entrez-6] 1 2 "Entrez Gene: PFKFB1 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1".

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PFKFB1

Related Research Articles

References

Further reading