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Phosphofructokinase Deficiency (PFK) - OFA What is Phosphofructokinase Deficiency (PFK)? PFK is an enzyme required for the metabolism of glucose into useable energy PFK deficiency is an inherited glycogen storage disease which causes hemolytic anemia and exertional myopathy
15. 4: Regulation of Glycolysis - Biology LibreTexts Phosphofructokinase-1 Regulation Recall that phosphofructokinase-1 (PFK1) mediates the third step in the glycolytic pathway with the conversion of fructose 6-phosphate to fructose 1,6-bisphosphate, as shown in Figure 15 4 6 15 4 6 The PFK1 reaction is the first irreversible reaction of glycolysis
Phosphofructokinase | Glycolysis, ATP, Enzymes | Britannica Phosphofructokinase, enzyme that is important in regulating the process of fermentation, by which one molecule of the simple sugar glucose is broken down to two molecules of pyruvic acid The enzyme, one of a class called transferases, catalyzes one of several specific reactions involved in this
Phosphofructokinase Deficiency (Glycogen Storage Disease) - Patient Phosphofructokinase (PFK) deficiency is a glycogen storage disorder (GSD) It is rare and is inherited as an autosomal recessive disorder There is a mutation in the gene encoding muscle PFK on chromosome 12 1 There is also reduced activity of red cell PFK
Balancing glycolytic flux: the role of 6-phosphofructo-2-kinase . . . The tetrameric enzyme PFK-1 catalyzes the conversion of Fru-6-P and ATP to fructose 1,6-bisphosphate and ADP (Figure 1) Interestingly, PFK-1 activity is inhibited by ATP, citrate or fatty acids, thereby adjusting glycolytic activity to environmental conditions and cellular metabolic demands