uu.seUppsala University Publications
Change search
ReferencesLink to record
Permanent link

Direct link
The contribution of Ca+ calmodulin activation of human erythrocyte AMP deaminase (isoform E) to the erythrocyte metabolic dysregulation of familial phosphofructokinase deficiency
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Clinical Chemistry. (biologisk struktur och funktion)
2006 (English)In: Haematologica, ISSN 0390-6078, Vol. 91, no 5, 652-5 p.Article in journal (Refereed) Published
Abstract [en]

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.
Keyword [en]
erythrocyte, purines, dysregulation, anemia
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-102297PubMedID: 16670071OAI: oai:DiVA.org:uu-102297DiVA: diva2:214602
Available from: 2009-05-06 Created: 2009-05-06 Last updated: 2009-07-29Bibliographically approved

Open Access in DiVA

No full text

By organisation
Clinical Chemistry
In the same journal
Medical and Health Sciences

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 170 hits
ReferencesLink to record
Permanent link

Direct link