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PFKM phosphofructokinase, muscle type. An ubiquitous metabolic enzyme involved in the synthesis and degradation of fructose 2,6-bisphosphate. Key control step of glycolysis. An allosteric enzyme activated by ADP, AMP, or fructose bisphosphate and inhibited by ATP or citrate. Activity: ATP D-fructose 6-phosphate = ADP D-fructose 1,6-bisphosphate. The holoenzyme consists of 4 subunits. The liver and muscle enzymes are homo-tetramers of four liver or muscle isoforms, respectively. The red blood cell enzyme consists hetero-tetramers of the muscle and liver isoforms. A subunit composition with a higher proportion of platelet type subunits is found in platelets, brain and fibroblasts. Defects in PFKM are the cause of glycogen storage disease VII (GSD-VII) also known as Tarui disease. Two alternatively spliced isoforms have been described. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - fructose and mannose; Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - pentose phosphate pathway; EC; Kinase, other
Chromosomal Location of Human Ortholog: 12q13.11
Cellular Component: 6-phosphofructokinase complex; apical plasma membrane; cytosol; extracellular exosome; nucleus
Molecular Function: 6-phosphofructokinase activity; ATP binding; fructose binding; identical protein binding; kinase binding; metal ion binding; protein binding; protein C-terminus binding; protein homodimerization activity
Biological Process: carbohydrate phosphorylation; fructose 6-phosphate metabolic process; glucose homeostasis; glycogen catabolic process; glycolytic process; muscle maintenance; positive regulation of insulin secretion; positive regulation of transcription from RNA polymerase II promoter; protein oligomerization
Disease: Glycogen Storage Disease Vii
Reference #:  P08237 (UniProtKB)
Alt. Names/Synonyms: 6-phosphofructo-1-kinase; 6-phosphofructokinase, muscle type; GSD7; K6PF; MGC8699; PFK-1; PFK-A; PFK1; PFKA; PFKM; PFKX; Phosphofructo-1-kinase isozyme A; Phosphofructokinase 1; phosphofructokinase, muscle; phosphofructokinase, polypeptide X; Phosphofructokinase-M; Phosphohexokinase
Gene Symbols: PFKM
Molecular weight: 85,183 Da
Basal Isoelectric point: 8.23  Predict pI for various phosphorylation states
CST Pathways:  Warburg Effect
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Protein Structure Not Found.
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