PFKFB1

6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1
PDB rendering based on 1c7z.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1K6M​
Identifiers
Symbols	PFKFB1 ; F6PK; HL2K; PFRX
External IDs	OMIM: 311790 MGI: 107816 HomoloGene: 105654 GeneCards: PFKFB1 Gene
EC number	2.7.1.105, 3.1.3.46
Gene ontology Molecular function • 6-phosphofructo-2-kinase activity • fructose-2,6-bisphosphate 2-phosphatase activity • protein binding • ATP binding • kinase binding • identical protein binding • fructose-6-phosphate binding Cellular component • cytosol • 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase complex Biological process • carbohydrate metabolic process • fructose metabolic process • fructose 2,6-bisphosphate metabolic process • glucose metabolic process • gluconeogenesis • glycolytic process • energy reserve metabolic process • protein dephosphorylation • organ regeneration • response to insulin • positive regulation of glucokinase activity • response to glucagon • intracellular signal transduction • response to starvation • small molecule metabolic process • carbohydrate phosphorylation • response to glucocorticoid • response to cAMP • canonical glycolysis Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	5207	18639
Ensembl	ENSG00000158571	ENSMUSG00000025271
UniProt	P16118	P70266
RefSeq (mRNA)	NM_001271804	NM_008824
RefSeq (protein)	NP_001258733	NP_032850
Location (UCSC)	Chr X: 54.93 – 55 Mb	Chr X: 150.59 – 150.64 Mb
PubMed search

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

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.^[2]

References

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

Further reading

• 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. 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. 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. 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.