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Increased red cell calcium, decreased calcium adenosine triphosphatase, and
altered membrane proteins during fava bean hemolysis in glucose-6-
phosphate dehydrogenase-deficient (Mediterranean variant) individuals
F Turrini, A Naitana, L Mannuzzu, G Pescarmona and P Arese
RBCs from four glucose-6-phosphate dehydrogenase (G6PD)-deficient
(Mediterranean variant) subjects were studied during fava bean hemolysis.
In the density-fractionated RBC calcium level, Ca2+-ATPase activity,
reduced glutathione level, and ghost protein pattern were studied. In the
bottom fraction, containing most heavily damaged RBCs, calcium level ranged
from 143 to 244 mumol/L RBCs (healthy G6PD- deficient controls: 17 +/- 5
mumol/L RBCs). The Ca2+-ATPase activity ranged from 0.87 to 1.84 mumol ATP
consumed/g Hb/min (healthy G6PD- deficient controls: 2.27 +/- 0.4). Sodium
dodecyl sulfate- polyacrylamide gel electrophoresis (SDS-PAGE) of ghosts
showed: (1) the presence of high mol wt aggregates (in three cases they
were reduced by dithioerythritol; in one case, only partial reduction was
possible); (2) the presence of multiple, scattered new bands; and (3) the
reduction of band 3. Oxidant-mediated damage to active calcium extrusion,
hypothetically associated with increased calcium permeability, may explain
the large increase in calcium levels. They, in turn, could activate
calcium-dependent protease activity, giving rise to the profound changes in
the ghost protein pattern.
Volume 66,
Issue 2,
pp. 302-305,
08/01/1985
Copyright © 1985 by The American Society of Hematology

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