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PMID:7492791
| Citation |
Joiner, CH, Franco, RS, Jiang, M, Franco, MS, Barker, JE and Lux, SE (1995) Increased cation permeability in mutant mouse red blood cells with defective membrane skeletons. Blood 86:4307-14 |
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| Abstract |
Cellular cation homeostasis in mouse erythrocytes with defective membrane skeletons was examined in three mouse mutants, hemolytic anemia (sphha/sphha), spherocytosis (sph/sph), and normoblastosis (nb/nb), and compared with reticulocytes produced by repetitive bleeding of congenic normal mice. To assess reticulocyte maturity, nucleic acid and transferrin receptor contents were measured by fluorescence flow cytometry; mutant cells were somewhat more mature than normal reticulocytes by these criteria. Red blood cell (RBC) sodium contents (Nac+) in homozygous sphha/sphha, sph/sph, and nb/nb animals were 30.1 +/- 0.9, 28.9 +/- 0.3, and 26.9 +/- 1.5 mmol/L cell, respectively, whereas cellular potassium (Kc+) was 102 +/- 2.6, 101 +/- 7.8, and 97.4 +/- 3.0. Nac+ and Kc+ in normal reticulocyte preparations were 11.3 +/- 0.7 and 123 +/- 10, respectively. Net Na+ and K+ fluxes in the presence of ouabain were markedly increased in mutant RBCs. Sodium uptake was 14.8 +/- 1.6, 15.4 +/- 3.3, and 14.7 +/- 3.1 mmol/L cell/h in sphha/sphha, sph/sph, and nb/nb mutants, respectively, whereas K+ loss was 17.0 +/- 4.0, 15.0 +/- 3.8, and 14.1 +/- 2.6. Normal mouse reticulocytes gained Na+ at a rate of 3.9 +/- 1.0 mmol/L cell/h and lost K+ at 6.0 +/- 2.1, rates indistinguishable from those in mature mouse RBCs. Potassium loss from sphha/sphha and nb/nb cells was not dependent on the presence of a Na+ gradient, and net cation movements were insensitive to bumetanide (sphha/sphha and nb/nb RBCs) and to chloride replacement with sulfamate (nb/nb cells). We conclude that mutant mouse RBCs with dysfunctional membrane skeletons have increased passive permeability to monovalent cations. These findings support a role of the membrane skeleton in the maintenance of the membrane permeability barrier and suggest that the abnormal permeability associated with human hereditary spherocytosis and elliptocytosis may be a consequence of the membrane skeleton defects reported in these disorders. |
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| Keywords |
Anemia, Hemolytic/blood; Anemia, Hemolytic/genetics; Animals; Biological Transport, Active; Cytoskeleton/drug effects; Erythroblastosis, Fetal/blood; Erythroblastosis, Fetal/genetics; Erythrocyte Membrane/metabolism; Homeostasis; Humans; Infant, Newborn; Mice; Mice, Mutant Strains; Permeability; Potassium/blood; Reticulocytes/metabolism; Sodium/blood; Sodium-Potassium-Exchanging ATPase/blood; Sodium-Potassium-Exchanging ATPase/genetics; Spherocytosis, Hereditary/blood; Spherocytosis, Hereditary/genetics |
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