Hemoglobin CC disease: rheological properties of erythrocytes and abnormalities in cell water

Murphy, John R.

doi:10.1172/jci105842

Cited by 41 publications

(13 citation statements)

References 24 publications

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“…This observation is supportive of earlier studies by Charache et al (25) and by Murphy (26), showing cellular dehydration in this disorder. We found no evidence for cells with a reduced S/V.…”

supporting

confidence: 93%

“…However, when the osmolality was reduced to 100 mosmol/kg, the deformability was restored to a virtually normal maximum value in all subpopulations. This evidence for cellular dehydration, which is well known to be characteristic of hemoglobin CC cells (25,26) creasing osmolality expected for high MCHC cells. The minimal changes in the initial slope of the DI curves were no greater than those seen for LPC-treated model cells.…”

mentioning

confidence: 66%

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Analysis of factors regulating erythrocyte deformability.

Mohandas¹,

Clark²,

Jacobs³

et al. 1980

J. Clin. Invest.

344

211

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supporting

confidence: 93%

mentioning

confidence: 66%

Analysis of factors regulating erythrocyte deformability.

Mohandas¹,

Clark²,

Jacobs³

et al. 1980

J. Clin. Invest.

344

211

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“…2). This is in agreement with the well known increase in MCHC described in Hb CC cells (5,11). It is appropriate to note that RBC We have recently reported the use ofthe T2 relaxation of P-31 in 2,3-DPG to detect polymerization of hemoglobin S in intact cells (9).…”

Section: Discussionsupporting

confidence: 89%

“…These erythrocytes have been found to be less filterable (4) and to exhibit equally elevated viscosity in both the oxy-and deoxygenated states as compared to Hb AA cells (5,10,11). In a previous publication (11), we described the use of the perfused rat mesoappendix to study flow properties of abnormal erythrocytes.…”

mentioning

confidence: 99%

Some aspects of the pathophysiology of homozygous Hb CC erythrocytes.

Fabry¹,

Kaul²,

Raventós³

et al. 1981

J. Clin. Invest.

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“…to be higher, if the cation content of CC and AA cells were the same. However, Murphy (2,3) reported that the cation and water content of CC cells is reduced, but did not investigate how cation transport in CC cells produces this reduced cation and water content. The experiments described in this paper were designed to answer that question.…”

mentioning

confidence: 99%

Regulation of cation content and cell volume in hemoglobin erythrocytes from patients with homozygous hemoglobin C disease.

Brugnara¹,

Kopin²,

Bunn³

et al. 1985

J. Clin. Invest.

168

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IntroductionErythrocytes from patients with homozygous hemoglobin C disease (CC cells) contain less K, Na, and water than do erythrocytes from normal subjects that contain only hemoglobin A (AA cells). In this paper, we provide evidence that the reduced K content and volume of CC cells are due to the activity in these but not in AA cells of a K transport system that is: (a) In this paper we examine the transport pathways for Na and K in CC erythrocytes. We show that CC cells regulate their volume through a ouabain-and bumetanide-insensitive pathway for K. This pathway seems to be responsible for the reduction in K and water content observed in CC cells. The pathway is dependent on cell volume and internal pH, and does not share any of the properties of the Ca-activated K transport system first described by Gardos (for a review see reference 5).A preliminary report of this work has previously appeared in another publication (6). Freedman and Hoffman (7).When the experiments were not performed on the same day of collection, the erythrocytes were stored at 4VC (20% hematocrit) in a preservation solution containing 140 mM KCI, 10 mM NaCI, 1 mM MgCl2, 2.5 mM NaH2PO4 buffer, pH 7.4, and 10 mM glucose. All experiments were performed within 2 d of sampling.Measurement of K efflux from fresh cells. K efflux from CC cells and controls was measured into Na or choline medium, as a function of external pH (pH.), at constant osmolarity (295-300 mosM), and as a function of osmolarity, at constant pHo (7.4). When the pH. was varied, the medium contained 140 mM NaCl, (or 140 mM choline chloride), I mM MgCI2, 10 mM gluc6oe, 0.1 mM ouabain, 0.01 mM bumetanide, and 10 mM Tris-MOPS, pH 6.2-8.0, at 37°C. When the osmolarity was varied, the medium contained 100 mM NaCl (or 100 mM choline chloride), I mM MgCl2, 10 mM Tris-MOPS, pH 7.4, at 37°C, 10 mM glucose, 0.1 mM ouabain, and 0.01 mM bumetanide. The osmolarity was varied from 220 to 400 mosM by adding choline chloride. For the flux assay, 0.4 ml of cell suspension in choline washing solution (hematocrit -30%) was added to 9 ml of flux medium, and this flux suspension was then distributed into six previously chilled 5-ml tubes. After capping, three tubes were incubated for 5 min and three for 25 min in a shaking water bath at 37°C. The time course of K efflux into hypotonic, isotonic, and hypertonic media was determined in preliminary experiments. A 25-min incubation was chosen in order to meet conditions of linear initial rate in the flux measurement. At the end of the incubation, the tubes were transferred to an ice bath and, after 2 min, they were spun at 3,000 g for 5 min. The supernatant was removed and the K concentration was measured by atomic absorption using standards for K with the same composition of the flux medium.Measurement of unidirectional radioactive influx in fresh cells. 9 ml of medium was chilled and mixed with 10 ACi of 22Na, or 30 Ci of 86Rb (86Rb was used as a tracer for K fluxes). The radioactivity in five aliquots of 20 Ml of medium was measured f...

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Hemoglobin CC disease: rheological properties of erythrocytes and abnormalities in cell water

Cited by 41 publications

References 24 publications

Analysis of factors regulating erythrocyte deformability.

Analysis of factors regulating erythrocyte deformability.

Some aspects of the pathophysiology of homozygous Hb CC erythrocytes.

Regulation of cation content and cell volume in hemoglobin erythrocytes from patients with homozygous hemoglobin C disease.

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