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Progressive inhibition of the Ca pump and Ca : Ca exchange in sickle red cells

Abstract

Sickle cell anaemia red cells (SS) were reported to have a high Ca content and an increased Ca uptake on deoxygenation1,2 but their Ca-pump activity was described as normal2. This seemed puzzling because the saturated Ca-extrusion rate of the normal, high Ca-affinity Ca pump is about 10 mmol per 1 cells per h (refs 3, 4) and the highest sickling-induced Ca influx reported in SS cells2,5 and observed in ATP-depleted sickle-trait (SA) red cells6 never exceeded 0.2 mmol per 1 cells per h. Normal pump performance is, therefore, incompatible with Ca accumulation unless SS cells have abnormally high Ca-binding capacity. We provide here evidence which suggests that SS cells have normal Ca-buffering capacity and probably genetically normal Ca pumps, but that the sickling process causes progressive Ca-pump failure and a marked reduction in Ca:Ca exchange.

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Bookchin, R., Lew, V. Progressive inhibition of the Ca pump and Ca : Ca exchange in sickle red cells. Nature 284, 561–563 (1980). https://doi.org/10.1038/284561a0

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