Ultracentrifugal analysis of the junction complexes of the red cell membrane cytoskeletal network: application to hereditary spherocytosis and metabolically depleted cells

1989 ◽  
Vol 59 (4) ◽  
pp. 385-389 ◽  
Author(s):  
Stephen A. Morris ◽  
Maud Kaufman
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Cytoskeletal Network ◽  
Network Application ◽  
Ultracentrifugal Analysis

scholarly journals Erythrocyte cellular and membrane deformability in hereditary spherocytosis

Blood ◽  
1979 ◽  
Vol 53 (3) ◽  
pp. 481-485 ◽  
Author(s):  
K Nakashima ◽  
E Beutler
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Volume Ratio ◽  
Osmotic Fragility ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Relative Rigidity ◽  
Altered Surface ◽  
Red Cell Membranes ◽  
Expected Increase

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.

scholarly journals Erythrocyte cellular and membrane deformability in hereditary spherocytosis

Blood ◽  
1979 ◽  
Vol 53 (3) ◽  
pp. 481-485 ◽  
Author(s):  
K Nakashima ◽  
E Beutler
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Volume Ratio ◽  
Osmotic Fragility ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Relative Rigidity ◽  
Altered Surface ◽  
Red Cell Membranes ◽  
Expected Increase

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.

Hereditary spherocytosis, elliptocytosis, and other red cell membrane disorders

Blood Reviews ◽  
2013 ◽  
Vol 27 (4) ◽  
pp. 167-178 ◽  
Author(s):  
Lydie Da Costa ◽  
Julie Galimand ◽  
Odile Fenneteau ◽  
Narla Mohandas
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Red Cell ◽  
Red Cell Membrane

scholarly journals Diminished osmotic fragility of human erythrocytes following the membrane insertion of oxygenated sterol compounds

Blood ◽  
1981 ◽  
Vol 58 (2) ◽  
pp. 317-325
Author(s):  
RA Streuli ◽  
JR Kanofsky ◽  
RB Gunn ◽  
S Yachnin
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Osmotic Fragility ◽  
Human Erythrocytes ◽  
Fragility Curve ◽  
Membrane Insertion ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Mean Cell Volume ◽  
Osmotic Lysis

Oxygenated sterol compounds (OSC), when incubated for 1 hr with human erythrocytes in lipoprotein-depleted medium at concentrations of 0.625- 5 X 10(-5) M, are inserted into the cell membrane and remain there despite subsequent washing of the cells. The insertion results in expansion of the surface area of the red cell ghost membrane, an increase in critical hemolytic volume, and as a consequence, in diminished osmotic fragility of the erythrocytes. This effect is seen with echinocyte-forming as well as with non-echinocyte-forming OSC. Erythrocytes treated with OSC do not differ from control cells with respect to their mean cell volume (MCV) in isotonic solution, water content, ion fluxes, and filterability through polycarbonate filters. The shift of the osmotic fragility curve toward lower NaCl concentrations is proportional to the amount of OSC inserted into the red cell membrane. 7 beta-Hydroxycholesterol, 22-ketocholesterol, and 20 alpha-hydroxycholesterol are the most potent inhibitors of osmotic lysis. The effect of OSC on osmotic fragility is diminished if the erythrocytes are incubated in a lipoprotein-containing medium; free cholesterol, however, does not change this effect. Various progesterones also protect red cell from osmotic lysis, but only if the erythrocytes are directly exposed to the compounds present in the hypotonic NaCl solutions used for measurement of their osmotic fragility. Progesterones do not remain in the membrane after the cells have been washed. The OSC are also capable of correcting the osmotic fragility curve of red cells from patients with hereditary spherocytosis. These experiments may suggest an approach to the pharmacologic treatment of hereditary spherocytosis.

scholarly journals Diminished osmotic fragility of human erythrocytes following the membrane insertion of oxygenated sterol compounds

Blood ◽  
1981 ◽  
Vol 58 (2) ◽  
pp. 317-325 ◽  
Author(s):  
RA Streuli ◽  
JR Kanofsky ◽  
RB Gunn ◽  
S Yachnin
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Osmotic Fragility ◽  
Human Erythrocytes ◽  
Fragility Curve ◽  
Membrane Insertion ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Mean Cell Volume ◽  
Osmotic Lysis

Abstract Oxygenated sterol compounds (OSC), when incubated for 1 hr with human erythrocytes in lipoprotein-depleted medium at concentrations of 0.625- 5 X 10(-5) M, are inserted into the cell membrane and remain there despite subsequent washing of the cells. The insertion results in expansion of the surface area of the red cell ghost membrane, an increase in critical hemolytic volume, and as a consequence, in diminished osmotic fragility of the erythrocytes. This effect is seen with echinocyte-forming as well as with non-echinocyte-forming OSC. Erythrocytes treated with OSC do not differ from control cells with respect to their mean cell volume (MCV) in isotonic solution, water content, ion fluxes, and filterability through polycarbonate filters. The shift of the osmotic fragility curve toward lower NaCl concentrations is proportional to the amount of OSC inserted into the red cell membrane. 7 beta-Hydroxycholesterol, 22-ketocholesterol, and 20 alpha-hydroxycholesterol are the most potent inhibitors of osmotic lysis. The effect of OSC on osmotic fragility is diminished if the erythrocytes are incubated in a lipoprotein-containing medium; free cholesterol, however, does not change this effect. Various progesterones also protect red cell from osmotic lysis, but only if the erythrocytes are directly exposed to the compounds present in the hypotonic NaCl solutions used for measurement of their osmotic fragility. Progesterones do not remain in the membrane after the cells have been washed. The OSC are also capable of correcting the osmotic fragility curve of red cells from patients with hereditary spherocytosis. These experiments may suggest an approach to the pharmacologic treatment of hereditary spherocytosis.

Reduction of in Vitro Autohemolysis in Hereditary Spherocytosis by Impermeant Molecules

Blood ◽  
1967 ◽  
Vol 30 (4) ◽  
pp. 449-456 ◽  
Author(s):  
DANIEL N. MOHLER ◽  
Norman Eby
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Red Cell ◽  
Atp Content ◽  
Red Cell Membrane ◽  
Phosphorylated Compounds ◽  
Osmotic Behavior

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.

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