Phosphofructokinase-1, Muscle Type

**Definition:** An allosteric enzyme that regulates glycolysis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-1,6-bisphosphate. In humans, PHOSPHOFRUCTOKINASE-1 in muscle exists as the homotetramer of M subunits. Defects in this muscle enzyme cause GLYCOGEN STORAGE DISEASE TYPE VII, also known as Tarui's disease.

**Tree numbers:** - D08.811.913.696.620.225.850.500.500

**Synonyms:** - Phosphofructo-1-kinase Isozyme A - 6-Phosphofructokinase, Muscle Type