Phosphofructokinase-1 liver type (PFKL)

Phosphofructokinase-1 liver type (PFKL) is a tetrameric enzyme and the liver isoform of phosphofructokinase-1, catalyzing the phosphorylation of D-fructose 6-phosphate to D-fructose 1,6-bisphosphate—the key, irreversible rate-limiting step of glycolysis[1][4][5][7]. PFKL activity is critical for regulating cell energy balance, metabolic switching, and the oxidative stress response. It is subject to complex allosteric regulation and post-translational modification, assembling into filaments in a substrate-dependent manner in cells[2][5]. Dysregulation or mutation of PFKL is implicated in glycogen storage diseases (such as Tarui disease), cancer metabolic reprogramming, and altered function in Down syndrome[1][4][7]. In immune cells, PFKL modulates NADPH production and reactive oxygen species generation. While no clinically used drugs directly target PFKL, its pivotal regulatory role in metabolism makes it a potential therapeutic target for metabolic diseases and cancer[4].

Other names

ATP-dependent 6-phosphofructokinase, liver typeATP-PFKPFK-LPFK-B6-phosphofructokinase type BPhosphofructo-1-kinase isozyme BPhosphohexokinasephosphofructokinase, liverliver-type 1-phosphofructokinase

Allosteric activation or inhibition of glycolytic flux (by phosphorylation or small-molecule effectors) - Modulation of enzyme stability (ubiquitination/deubiquitination)