You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Identification
HMDB Protein ID HMDBP00799
Secondary Accession Numbers
  • 6079
Name 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase 1
Synonyms
  1. 6-phosphofructo-2-kinase
  2. 6PF-2-K/Fru-2,6-P2ase 1
  3. 6PF-2-K/Fru-2,6-P2ase liver isozyme
  4. Fructose-2,6-bisphosphatase
  5. PFK/FBPase 1
Gene Name PFKFB1
Protein Type Enzyme
Biological Properties
General Function Involved in catalytic activity
Specific Function Synthesis and degradation of fructose 2,6-bisphosphate.
Pathways
  • Fructose and Mannose Degradation
  • Fructose and mannose metabolism
  • Fructose intolerance, hereditary
  • Fructosuria
  • HIF-1 signaling pathway
Reactions
D-Fructose 2,6-bisphosphate + Water → Fructose 6-phosphate + Phosphate details
Adenosine triphosphate + Fructose 6-phosphate → ADP + D-Fructose 2,6-bisphosphate details
D-Fructose 2,6-bisphosphate + Water → Beta-D-Fructose 6-phosphate + Phosphate details
GO Classification
Biological Process
small molecule metabolic process
fructose metabolic process
organ regeneration
response to glucagon stimulus
glycolysis
response to insulin stimulus
response to starvation
intracellular protein kinase cascade
gluconeogenesis
response to glucocorticoid stimulus
positive regulation of glucokinase activity
energy reserve metabolic process
fructose 2,6-bisphosphate metabolic process
response to cAMP
Cellular Component
6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1 complex
Function
binding
catalytic activity
transferase activity
transferase activity, transferring phosphorus-containing groups
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
adenyl ribonucleotide binding
atp binding
phosphotransferase activity, alcohol group as acceptor
phosphofructokinase activity
6-phosphofructo-2-kinase activity
Molecular Function
ATP binding
fructose-2,6-bisphosphate 2-phosphatase activity
6-phosphofructo-2-kinase activity
fructose-6-phosphate binding
Process
metabolic process
small molecule metabolic process
alcohol metabolic process
monosaccharide metabolic process
hexose metabolic process
fructose metabolic process
fructose 2,6-bisphosphate metabolic process
Cellular Location Not Available
Gene Properties
Chromosome Location X
Locus Xp11.21
SNPs PFKFB1
Gene Sequence
>1416 bp
ATGTCTCCAGAGATGGGAGAGCTCACCCAAACCAGGTTGCAGAAGATCTGGATTCCACAC
AGCAGCGGCAGCAGCAGGCTGCAACGGAGAAGGGGCTCATCCATACCCCAGTTTACCAAT
TCCCCCACAATGGTGATCATGGTGGGTTTACCAGCTCGAGGCAAGACCTATATCTCCACA
AAGCTCACACGATATCTCAACTGGATAGGAACACCAACTAAAGTGTTTAATTTAGGCCAG
TATCGACGAGAGGCAGTGAGCTACAAGAACTATGAATTCTTTCTTCCAGACAACATGGAA
GCCCTGCAAATCAGGAAGCAGTGCGCCCTGGCAGCCCTGAAGGATGTTCACAACTATCTC
AGCCATGAGGAAGGTCATGTTGCGGTTTTTGATGCCACCAACACTACCAGAGAACGACGG
TCACTGATCCTGCAGTTTGCAAAAGAACATGGTTACAAGGTGTTTTTCATTGAGTCCATT
TGTAATGACCCTGGCATAATTGCAGAAAACATCAGGCAAGTGAAACTTGGCAGCCCTGAT
TATATAGACTGTGACCGGGAAAAGGTTCTGGAAGACTTTCTAAAGAGAATTGAGTGCTAT
GAGGTCAACTACCAACCCTTGGATGAGGAACTGGACAGCCACCTGTCCTACATCAAGATC
TTCGACGTGGGCACACGCTACATGGTGAACCGAGTGCAGGATCACATCCAGAGCCGCACA
GTCTACTACCTCATGAATATCCATGTCACACCTCGCTCCATCTACCTTTGCCGACATGGC
GAGAGTGAACTCAACATCAGAGGCCGCATCGGAGGTGACTCTGGCCTCTCAGTTCGCGGC
AAGCAGTATGCCTATGCCCTGGCCAACTTCATTCAGTCCCAGGGCATCAGCTCCCTGAAG
GTGTGGACCAGTCGCATGAAGAGGACCATCCAGACAGCTGAGGCCCTGGGTGTCCCCTAT
GAGCAGTGGAAGGCCCTGAATGAGATTGATGCGGGTGTCTGTGAGGAGATGACCTATGAA
GAAATCCAGGAACATTACCCTGAAGAATTTGCACTGCGAGACCAAGATAAATATCGCTAC
CGCTATCCCAAGGGAGAGTCCTATGAGGATCTGGTTCAGCGTCTGGAGCCAGTGATAATG
GAGCTAGAACGACAGGAGAATGTACTGGTGATCTGCCACCAGGCTGTCATGCGGTGCCTC
CTGGCCTATTTCCTGGATAAAAGTTCAGATGAGCTTCCATATCTCAAGTGCCCTCTGCAC
ACAGTGCTCAAACTCACTCCTGTGGCTTATGGCTGCAAAGTGGAATCCATCTACCTGAAT
GTGGAGGCCGTGAACACACACCGGGAGAAGCCTGAGAATGTGGACATCACCCGGGAACCT
GAGGAAGCCCTGGATACTGTCCCAGCCCACTACTGA
Protein Properties
Number of Residues 471
Molecular Weight 54680.86
Theoretical pI 6.586
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1
MSPEMGELTQTRLQKIWIPHSSGSSRLQRRRGSSIPQFTNSPTMVIMVGLPARGKTYIST
KLTRYLNWIGTPTKVFNLGQYRREAVSYKNYEFFLPDNMEALQIRKQCALAALKDVHNYL
SHEEGHVAVFDATNTTRERRSLILQFAKEHGYKVFFIESICNDPGIIAENIRQVKLGSPD
YIDCDREKVLEDFLKRIECYEVNYQPLDEELDSHLSYIKIFDVGTRYMVNRVQDHIQSRT
VYYLMNIHVTPRSIYLCRHGESELNIRGRIGGDSGLSVRGKQYAYALANFIQSQGISSLK
VWTSHMKRTIQTAEALGVPYEQWKALNEIDAGVCEEMTYEEIQEHYPEEFALRDQDKYRY
RYPKGESYEDLVQRLEPVIMELERQENVLVICHQAVMRCLLAYFLDKSSDELPYLKCPLH
TVLKLTPVAYGCKVESIYLNVEAVNTHREKPENVDITREPEEALDTVPAHY
GenBank ID Protein 35503
UniProtKB/Swiss-Prot ID P16118
UniProtKB/Swiss-Prot Entry Name F261_HUMAN
PDB IDs
GenBank Gene ID X52638
GeneCard ID PFKFB1
GenAtlas ID PFKFB1
HGNC ID HGNC:8872
References
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  2. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  3. Ross MT, Grafham DV, Coffey AJ, Scherer S, McLay K, Muzny D, Platzer M, Howell GR, Burrows C, Bird CP, Frankish A, Lovell FL, Howe KL, Ashurst JL, Fulton RS, Sudbrak R, Wen G, Jones MC, Hurles ME, Andrews TD, Scott CE, Searle S, Ramser J, Whittaker A, Deadman R, Carter NP, Hunt SE, Chen R, Cree A, Gunaratne P, Havlak P, Hodgson A, Metzker ML, Richards S, Scott G, Steffen D, Sodergren E, Wheeler DA, Worley KC, Ainscough R, Ambrose KD, Ansari-Lari MA, Aradhya S, Ashwell RI, Babbage AK, Bagguley CL, Ballabio A, Banerjee R, Barker GE, Barlow KF, Barrett IP, Bates KN, Beare DM, Beasley H, Beasley O, Beck A, Bethel G, Blechschmidt K, Brady N, Bray-Allen S, Bridgeman AM, Brown AJ, Brown MJ, Bonnin D, Bruford EA, Buhay C, Burch P, Burford D, Burgess J, Burrill W, Burton J, Bye JM, Carder C, Carrel L, Chako J, Chapman JC, Chavez D, Chen E, Chen G, Chen Y, Chen Z, Chinault C, Ciccodicola A, Clark SY, Clarke G, Clee CM, Clegg S, Clerc-Blankenburg K, Clifford K, Cobley V, Cole CG, Conquer JS, Corby N, Connor RE, David R, Davies J, Davis C, Davis J, Delgado O, Deshazo D, Dhami P, Ding Y, Dinh H, Dodsworth S, Draper H, Dugan-Rocha S, Dunham A, Dunn M, Durbin KJ, Dutta I, Eades T, Ellwood M, Emery-Cohen A, Errington H, Evans KL, Faulkner L, Francis F, Frankland J, Fraser AE, Galgoczy P, Gilbert J, Gill R, Glockner G, Gregory SG, Gribble S, Griffiths C, Grocock R, Gu Y, Gwilliam R, Hamilton C, Hart EA, Hawes A, Heath PD, Heitmann K, Hennig S, Hernandez J, Hinzmann B, Ho S, Hoffs M, Howden PJ, Huckle EJ, Hume J, Hunt PJ, Hunt AR, Isherwood J, Jacob L, Johnson D, Jones S, de Jong PJ, Joseph SS, Keenan S, Kelly S, Kershaw JK, Khan Z, Kioschis P, Klages S, Knights AJ, Kosiura A, Kovar-Smith C, Laird GK, Langford C, Lawlor S, Leversha M, Lewis L, Liu W, Lloyd C, Lloyd DM, Loulseged H, Loveland JE, Lovell JD, Lozado R, Lu J, Lyne R, Ma J, Maheshwari M, Matthews LH, McDowall J, McLaren S, McMurray A, Meidl P, Meitinger T, Milne S, Miner G, Mistry SL, Morgan M, Morris S, Muller I, Mullikin JC, Nguyen N, Nordsiek G, Nyakatura G, O'Dell CN, Okwuonu G, Palmer S, Pandian R, Parker D, Parrish J, Pasternak S, Patel D, Pearce AV, Pearson DM, Pelan SE, Perez L, Porter KM, Ramsey Y, Reichwald K, Rhodes S, Ridler KA, Schlessinger D, Schueler MG, Sehra HK, Shaw-Smith C, Shen H, Sheridan EM, Shownkeen R, Skuce CD, Smith ML, Sotheran EC, Steingruber HE, Steward CA, Storey R, Swann RM, Swarbreck D, Tabor PE, Taudien S, Taylor T, Teague B, Thomas K, Thorpe A, Timms K, Tracey A, Trevanion S, Tromans AC, d'Urso M, Verduzco D, Villasana D, Waldron L, Wall M, Wang Q, Warren J, Warry GL, Wei X, West A, Whitehead SL, Whiteley MN, Wilkinson JE, Willey DL, Williams G, Williams L, Williamson A, Williamson H, Wilming L, Woodmansey RL, Wray PW, Yen J, Zhang J, Zhou J, Zoghbi H, Zorilla S, Buck D, Reinhardt R, Poustka A, Rosenthal A, Lehrach H, Meindl A, Minx PJ, Hillier LW, Willard HF, Wilson RK, Waterston RH, Rice CM, Vaudin M, Coulson A, Nelson DL, Weinstock G, Sulston JE, Durbin R, Hubbard T, Gibbs RA, Beck S, Rogers J, Bentley DR: The DNA sequence of the human X chromosome. Nature. 2005 Mar 17;434(7031):325-37. [PubMed:15772651 ]
  4. Lange AJ, Pilkis SJ: Sequence of human liver 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase. Nucleic Acids Res. 1990 Jun 25;18(12):3652. [PubMed:2163524 ]
  5. Algaier J, Uyeda K: 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. 1988 May 31;153(1):328-33. [PubMed:2837207 ]