The contribution of Ca+ calmodulin activation of human erythrocyte AMP deaminase (isoform E) to the erythrocyte metabolic dysregulation of familial phosphofructokinase deficiency
2006 (English)In: Haematologica, ISSN 0390-6078, Vol. 91, no 5, 652-5 p.Article in journal (Refereed) Published
Erythrocyte membrane leakage of Ca2+ in familial phosphofructokinase deficiency results in a compensatory increase of Ca2+-ATPase activity that depletes ATP and leads to diminished erythrocyte deformability and a higher rate of hemolysis. Lowered ATP levels in circulating erythrocytes are accompanied by increased IMP, indicating that activated AMP deaminase plays a role in this metabolic dysregulation. Exposure to a calmodulin antagonist significantly slows IMP accumulation during experimental energy imbalance in patients' cells to levels that are similar to those in untreated controls, implying that Ca2+-calmodulin is involved in erythrocyte AMP deaminase activation in familial phosphofructokinase deficiency. Therapies directed against activated isoform E may be beneficial in this compensated anemia.
Place, publisher, year, edition, pages
2006. Vol. 91, no 5, 652-5 p.
erythrocyte, purines, dysregulation, anemia
Medical and Health Sciences
IdentifiersURN: urn:nbn:se:uu:diva-102297PubMedID: 16670071OAI: oai:DiVA.org:uu-102297DiVA: diva2:214602