FunctionPlays a major role in regulating hemoglobin oxygen affinity by controlling the levels of 2,3-bisphosphoglycerate (2,3-BPG). Also exhibits mutase (EC 220.127.116.11) and phosphatase (EC 18.104.22.168) activities.
Involvement in diseaseDefects in BPGM are the cause of bisphosphoglycerate mutase deficiency (BPGMD) [MIM:222800]. A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis.
Sequence similaritiesBelongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily.
Post-translational modificationsGlycation of Lys-159 in diabetic patients inactivates the enzyme.