IN VITRO PROTEOLYSIS OF RED CELL MEMBRANE IN HEREDITARY SPHEROCYTOSIS

1992 ◽  
Vol 81 (2) ◽  
pp. 312-313
Author(s):  
Ricardo Marques Costa ◽  
Gabriel Tamagnini
Blood ◽  
1967 ◽  
Vol 30 (4) ◽  
pp. 449-456 ◽  
Author(s):  
DANIEL N. MOHLER ◽  
Norman Eby

Abstract It has been shown that a variety of compounds will reduce the abnormal autohemolysis which occurs after 48 hours of incubation of HS erythrocytes. These compounds either provide energy in the form of ATP, as is the case with glucose and adenosine, or provide an external osmotic force by virtue of their inability to cross the red cell membrane, as is the case with phosphorylated compounds, glutathione and sucrose. None of these latter compounds caused a significant preservation of erythrocyte ATP content. In assessing the effect of added compounds in the autohemolysis test, their osmotic behavior should be taken into account.


1991 ◽  
Vol 79 (1) ◽  
pp. 108-112 ◽  
Author(s):  
M. C. Matteis ◽  
V. De Angelis ◽  
F. Sorrentino ◽  
E. Bonollo ◽  
L. Vettore

1992 ◽  
Vol 81 (2) ◽  
pp. 313-314
Author(s):  
L. Vettore ◽  
V. De Angelis ◽  
E. Bonollo ◽  
M. C. Matteis

Blood ◽  
1979 ◽  
Vol 53 (3) ◽  
pp. 481-485 ◽  
Author(s):  
K Nakashima ◽  
E Beutler

In order to determine whether the relative rigidity of the hereditary spherocytosis (HS) red cell is due to membrane rididity or merely to an altered surface/volume ratio, we investigated the deformability of resealed red cell membranes from patients with HS. Whereas the osmotic fragility of intact red cells of HS patients showed the expected increase, the osmotic fragility of resealed HS membranes was normal, thus indicating that their surface/volume ratio was normal. Measurements with an ektacytometer showed that deformability of intact HS cells was markedly diminished, whereas deformability of resealed HS membranes was normal. These findings indicate that the HS red cell membrane is not intrinsically abnormally rigid, as has been suggested, but that the lack of deformability of the erythrocyte is primarily a function of the altered surface/volume ratio.


Blood ◽  
1976 ◽  
Vol 47 (6) ◽  
pp. 909-917 ◽  
Author(s):  
TA Lane ◽  
ER Burka

Abstract Red blood cells exposed to cyanate (CNO) in vitro have a concentration- dependent decreased cell survival time associated with an inhibition of the ability of the cell membrane to synthesize lipids. The t1/2 of rabbit erythrocytes exposed to 30 mM or 50 mM cyanate for 1 hr at 37 degrees C is reduced from the normal 24 days to 15 and 9 days, respectively. The cyanate-induced defect in membrane lipid metabolism is irreversible. Carbamylation of membrane proteins and damage to metabolism are minimized by limiting exposure in vitro to 15 mM cyanate at 4 degrees C for 30 min. Cells carbamylated under these conditions do not have a shortened life span. Levels of globin carbamylation of 0.5 moles CNO/mole hemoglobin, shown to be clinically effective in prolonging the life span of sickle erythrocytes, are obtained under these conditions and reach maximal levels after only 30 min of incubation. Carbamylation of blood in CPD anticoagulant is inferior to either ACD or heparin. The findings indicate that adequate carbamylation of sickle erythrocytes with minimal red cell membrane damage can be achieved without significant modification of the standard plasmapheresis procedure utilized by the working blood bank.


Blood ◽  
1979 ◽  
Vol 53 (3) ◽  
pp. 481-485 ◽  
Author(s):  
K Nakashima ◽  
E Beutler

Abstract In order to determine whether the relative rigidity of the hereditary spherocytosis (HS) red cell is due to membrane rididity or merely to an altered surface/volume ratio, we investigated the deformability of resealed red cell membranes from patients with HS. Whereas the osmotic fragility of intact red cells of HS patients showed the expected increase, the osmotic fragility of resealed HS membranes was normal, thus indicating that their surface/volume ratio was normal. Measurements with an ektacytometer showed that deformability of intact HS cells was markedly diminished, whereas deformability of resealed HS membranes was normal. These findings indicate that the HS red cell membrane is not intrinsically abnormally rigid, as has been suggested, but that the lack of deformability of the erythrocyte is primarily a function of the altered surface/volume ratio.


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