Abstract
Extract: ATPase activity and monovalent cation transport were examined in erythrocytes (RBC) of subjects with cystic fibrosis (CF). The RBC membrane (Ga2+)ATPase activity of homozygous patients and obligate heterozygotes was indistinguishable from that of normal control subjects. This was true similarly for total, ouabain-sensitive, and ouabain-insensitive (Na+-K+)ATPase.
Total, ouabain-sensitive, and ouabain-insensitive 24Na+ transport were normal in CF cells. Ouabain-induced Na+ accumulation was similar in CF and normal RBG. Measurements of K+ transport in CF RBC were, likewise, normal.
We conclude that intrinsic ATPase and monovalent cation transport functions are normal in CF RBC. We were unable to confirm several abnormalities in these cells reported previously. The GF RBC is not helpful in defining a transport abnormality in that entity.
Speculation: These studies document normal intrinsic transport function in RBC of patients with CF. There have been recent reports of an abnormal serum factor in patients with CF. This factor might be involved in the production of a transport defect in CF. Further studies are indicated to determine whether CF plasma contains a factor capable of altering membrane transport functions of normal RBC.
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Feig, S., Segel, G., Kern, K. et al. Erythrocyte Transport Function in Cystic Fibrosis. Pediatr Res 8, 594–597 (1974). https://doi.org/10.1203/00006450-197405000-00008
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DOI: https://doi.org/10.1203/00006450-197405000-00008
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