Sickle cell anemia is a multi organ system disease caused by the abnormal physical properties of HbS. HbS has the unique tendency to polymerize and distort RBC in the circulation, resulting in widespread vascular insufficiency. The polymerization (and sickling) tendency of HbS- containing RBCs is exponentially related to the concentration of HbS in the cell. Sickling itself causes the cell to lose water and KCL, further exacerbating sickling as cells become dehydrated. RBC Mg plays a central role in the control of cell water content, volume and membrane functions in normal and sickle RBCs. K-Cl cotransport, a system involved in dehydration of sickle RBCs, is markedly inhibited by increasing cell Mg. There are reports which claim that Mg content of dense fraction of SS cells may be decreased. Dietary intake of Mg in SS patients has not been studied.
The specific aims of this proposal are the following: 1) Assess the dietary intake of Mg in patients with SS disease; 2) study the regulation of RBC Mg and the effects of Mg on ion transport and other membrane functions in normal and sickle erythrocytes and in genetically determined high Mg and low MG mouse erythrocytes; 3) assess the effect of dietary Mg supplementation in SS patients.
| De Franceschi, Lucia; Biondani, Andrea; Carta, Franco et al. (2008) PTPepsilon has a critical role in signaling transduction pathways and phosphoprotein network topology in red cells. Proteomics 8:4695-708 |
| De Franceschi, Lucia; Daraio, Filomena; Filippini, Alida et al. (2006) Liver expression of hepcidin and other iron genes in two mouse models of beta-thalassemia. Haematologica 91:1336-42 |
| Rivera, Alicia; De Franceschi, Lucia; Peters, Luanne L et al. (2006) Effect of complete protein 4.1R deficiency on ion transport properties of murine erythrocytes. Am J Physiol Cell Physiol 291:C880-6 |
| De Franceschi, Lucia; Villa-Moruzzi, Emma; Biondani, Andrea et al. (2006) Regulation of K-Cl cotransport by protein phosphatase 1alpha in mouse erythrocytes. Pflugers Arch 451:760-8 |
| Rivera, Alicia; Ferreira, Ana; Bertoni, Danielle et al. (2005) Abnormal regulation of Mg2+ transport via Na/Mg exchanger in sickle erythrocytes. Blood 105:382-6 |
| Mallozzi, Cinzia; De Franceschi, Lucia; Brugnara, Carlo et al. (2005) Protein phosphatase 1alpha is tyrosine-phosphorylated and inactivated by peroxynitrite in erythrocytes through the src family kinase fgr. Free Radic Biol Med 38:1625-36 |
| De Franceschi, Lucia; Rivera, Alicia; Fleming, Mark D et al. (2005) Evidence for a protective role of the Gardos channel against hemolysis in murine spherocytosis. Blood 106:1454-9 |
| de Franceschi, Lucia; Turrini, Franco; Honczarenko, Marek et al. (2004) In vivo reduction of erythrocyte oxidant stress in a murine model of beta-thalassemia. Haematologica 89:1287-98 |
| Begenisich, Ted; Nakamoto, Tesuji; Ovitt, Catherine E et al. (2004) Physiological roles of the intermediate conductance, Ca2+-activated potassium channel Kcnn4. J Biol Chem 279:47681-7 |
| De Franceschi, L; Olivieri, O; Corrocher, R (2004) Erythrocyte aging in neurodegenerative disorders. Cell Mol Biol (Noisy-le-grand) 50:179-85 |
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