D-fructose 6-phosphate + ATP => D-fructose 1,6-bisphosphate + ADP

Stable Identifier
R-HSA-70467
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Cytosolic phosphofructokinase 1 catalyzes the reaction of fructose 6-phosphate and ATP to form fructose 1,6-bisphosphate and ADP. This reaction, irreversible under physiological conditions, is the rate limiting step of glycolysis. Phosphofructokinase 1 activity is allosterically regulated by ATP, citrate, and fructose 2,6-bisphosphate.

Phosphofructokinase 1 is active as a tetramer (although higher order multimers, not annotated here, may form in vivo). Two isoforms of phosphofructokinase 1 monomer, L and M, are widely expressed in human tissues. Different tissues can contain different homotetramers or heterotetramers: L4 in liver, M4 in muscle, and all possible heterotetramers, L4, L3M, L2M2, LM3, and M4, in red blood cells, for example (Raben et al. 1995; Vora et al. 1980, 1987; Vora 1981). A third isoform, P, is abundant in platelets, where it is found in P4, P3L, P2L2, and PL3 tetramers (Eto et al. 1994; Vora et al. 1987).

Literature References
PubMed ID Title Journal Year
6444721 Isozymes of human phosphofructokinase: identification and subunit structural characterization of a new system

Vora, S, Seaman, C, Durham, S, Piomelli, S

Proc Natl Acad Sci U S A 1980
6451249 Isozymes of human phosphofructokinase in blood cells and cultured cell lines: molecular and genetic evidence for a trigenic system

Vora, S

Blood 1981
7825568 Functional expression of human mutant phosphofructokinase in yeast: genetic defects in French Canadian and Swiss patients with phosphofructokinase deficiency

Raben, N, Exelbert, R, Spiegel, R, Sherman, JB, Nakajima, H, Plotz, P, Heinisch, J

Am J Hum Genet 1995
8117307 Cloning of a complete protein-coding sequence of human platelet-type phosphofructokinase isozyme from pancreatic islet.

Eto, K, Sakura, H, Yasuda, K, Hayakawa, T, Kawasaki, E, Moriuchi, R, Nagataki, S, Yazaki, Y, Kadowaki, T

Biochem Biophys Res Commun 1994
2960695 Characterization of the enzymatic defect in late-onset muscle phosphofructokinase deficiency. New subtype of glycogen storage disease type VII.

Vora, S, DiMauro, S, Spear, D, Harker, D, Danon, MJ

J Clin Invest 1987
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Catalyst Activity
Title
6-phosphofructokinase activity of PFK tetramer [cytosol]
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