The contribution of Ca+ calmodulin activation of human erythrocyte AMP deaminase (isoform E) to the erythrocyte metabolic dysregulation of familial phosphofructokinase deficiency.

Abstract	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.
Authors	Richard L Sabina, Anders Waldenström, Gunnar Ronquist
Journal	Haematologica (Haematologica) Vol. 91 Issue 5 Pg. 652-5 (May 2006) ISSN: 1592-8721 [Electronic] Italy
PMID	16670071 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	Calmodulin Isoenzymes Inosine Monophosphate Hypoxanthine p-Methoxy-N-methylphenethylamine Adenosine Triphosphate AMP Deaminase Calcium-Transporting ATPases Calcium
Topics	AMP Deaminase (blood) Adenosine Triphosphate (biosynthesis, blood) Anemia, Hemolytic, Congenital (blood, enzymology, etiology) Calcium (physiology) Calcium-Transporting ATPases (blood) Calmodulin (antagonists & inhibitors, blood) Cell Membrane Permeability Enzyme Activation Erythrocyte Deformability Erythrocytes (enzymology) Glycogen Storage Disease Type VII (blood, genetics) Glycolysis Humans Hypoxanthine (blood) Inosine Monophosphate (blood) Isoenzymes (blood) Models, Biological p-Methoxy-N-methylphenethylamine (pharmacology)

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