基因名:
PKLR
产品别名:
PK1; PKL; PKRL; RPK; PKLR; pyruvate kinase L/R; pyruvate kinase L/R; pyruvate kinase PKLR; R-type/L-type pyruvate kinase; pyruvate kinase 1; pyruvate kinase isozyme R/L; pyruvate kinase isozymes L/R; pyruvate kinase isozymes R/L; pyruvate kinase type L; pyruvate kinase, liver and RBC; pyruvate kinase, liver and blood cell; red cell/liver pyruvate kinase; 丙酮酸激酶同工酶R/L(PKLR); 丙酮酸激酶肝型;
背景信息:
Pyruvate Kinase Isozymes R/L (PKLR) belongs to the pyruvate kinase family, 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; M2 is found in early fetal tissues. PKLR exists as a homotetramer and catalyzes the production of phosphoenolpyruvate from pyruvate and ATP. Defects in PKLR are also the cause of pyruvate kinase deficiency of red cells, which is a frequent cause of hereditary non-spherocytic hemolytic anemia.