catalytic activity:ATP + pyruvate = ADP + phosphoenolpyruvate.,cofactor:Divalent metal cations.,cofactor:Magnesium.,cofactor:Potassium.,disease:Defects in PKLR are a cause of chronic nonspherocytic hemolytic anemia (CNSHA) [MIM:266200]; also called hereditary nonspherocytic hemolytic anemia (HNSHA).,disease:Defects in PKLR are the cause of pyruvate kinase hyperactivity [MIM:102900]; also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP.,miscellaneous:There are 4 isozymes of pyruvate kinase in mammals: L, R, M1 and M2. L type is major isozyme in the liver, R is found in red cells, M1 is the main form in muscle, heart and brain, and M2 is found in early fetal tissues.,online information:Pyruvate kinase entry,pathway:Carbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 5/5.,similarity:Belongs to the pyruvate kinase family.,subunit:Homotetramer.,
cytosol,extracellular exosome,
Epithelium,Pancreas,