Erythrocyte Enigmas in Cystic Fibrosis

Hadden, John W.; Hansen, Leland G.; Shapiro, Burton L.; Warwick, Warren J.

doi:10.3181/00379727-142-37070

Cited by 9 publications

(5 citation statements)

References 4 publications

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“…Steady-state monovalent cation contents were not different in CF RBC from normal RBC, confirming the findings of other investigators (10,11). It is especially important to note that cellular Na is normal in CF cells because this allows analysis of external NaK pump kinetics with the assurance that the degree of pump activation by internal Na is the same in CF and normal cells.…”

Section: Resultssupporting

confidence: 91%

“…The portion of Na efflux inhibited by ethacrinic acid (which was later found to block NaK cotransport) was reported by Balfe et al (8) to be reduced in CF RBC, although Lapey and Gardner (9) observed this effect in male and not female CF patients, a phenomenon inconsistent with a single autosomal gene defect. Hadden et al (10) found normal ethacrinic acid inhibitable 86Rb uptake in CF RBC. Interpretation of these studies is hampered by the fact that the NaK cotransport system was not well understood at the time they were published.…”

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confidence: 96%

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Kinetic Parameters of Rubidium Transport Pathways Are Normal in Cystic Fibrosis Red Cells

Joiner

1988

Pediatr Res

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ABSTRACT. The abnormalities in ion transport in cystic fibrosis (CF) respiratory and sweat duct epithelia have prompted Studies of ion permeability in CF red blood cells (RBC) although previous reports have been contradictory. In this study, the kinetic characteristics of the three major cation transport systems in RBC were evaluated by measuring rubidium (Rb) uptake at various external Rb concentrations. The maximal velocity and affinity for external Rb (KH) of the NaK pump were normal in CF RBC, as were the maximal velocity and K, for Rb of the NaK cotransport system. Residual (ouabain and bumetanide insensitive) Rb uptake, and steady state RBC Na and K contents were also normal. These data indicate the NaK pump and cotransport system do not exhibit primary or secondary perturbations in CF RBC, and suggest that the noncarrier-mediated membrane permeability to cations is also normal in these cells. (Pediatr Res 24: 495-498, 1988) Abbreviations CF, cystic fibrosis RBC, red blood cell V , , maximal velocity K, Rb, external K or Rb PBS, phosphate-buffered saline OS, ouabain-sensitive Co, cotransport CF is an autosomal recessive disorder manifesting multiple exocrine abnormalities (1). The diagnostically important elevation of sweat chloride reflects malfunctioning of the salt resorbing mechanisms of sweat duct epithelium. Recent work has indicated that the basis of this dysfunction is low C1 permeability of the apical membrane of the ductal epithelial cells (2). Respiratory epithelia share this defect (3), which appears in these cells to arise from abnormalities of the adrenergic regulatory mechanisms of the C1 channel (4-6). Other recent studies of CF respiratory epithelia have also shown increased permeability of the apical membrane to Na, accompanied by elevated Na pump rates at the basolateral membrane, with increased numbers of [3H]ouabain binding sites and ouabain-inhibitable oxygen consumption (7). These findings suggest that, in addition to the C1 channel,

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Section: Resultssupporting

confidence: 91%

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confidence: 96%

Kinetic Parameters of Rubidium Transport Pathways Are Normal in Cystic Fibrosis Red Cells

Joiner

1988

Pediatr Res

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“…Hadden et al [6] have recently reported totally normal S6Rb and SZSr transport in CF erythrocytes. Because R b + and Sr" are transported by the same mechanisms as Na+ and Ca2+, respectively, they inferred that Na+ and Ca2+ transport is normal in CF cells.…”

Section: Introductionmentioning

confidence: 94%

Erythrocyte Transport Function in Cystic Fibrosis

et al. 1974

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“…While Balfe et al (2) found the activities of ouabain-sensitive ATPase of CF erythrocyte membranes to be decreased by about 30%, subsequent investigators (7,16,22,27,32,35) have observed that any decreased (Na + K)-ATPase activities are not significantly different from control activities. Our finding of a marginally significant decrease in the pancreatic insufficient subpopulation probably reflects technical and perhaps patient population differences from these studies.…”

Section: Discussionmentioning

confidence: 94%

Calcium-ATPase Activity in Cystic Fibrosis Erythrocyte Membranes: Decreased Activity in Patients with Pancreatic Insufficiency

et al. 1984

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Summary modulin, of the pancreatic sufficient patients (34.3 f 4.2 and 75.9 f 6.9 nmol/min/mg) was indistinguishable from that of The activity of Ca-ATPase (Ca2+,Mg2+-ATPase, ATP phos-controls (35.8 2.6 and 84.3 f 4.7 nmol/min/mg), while both phohydrolase, EC 3.6.1.3) was measured in erythrocyte mem-activities of patients with pancreatic insufficiency were signifibrane preparations from 37 cystic fibrosis patients, 27 with cantly decreased (28.9 + 1.3, < 0.02; 65.2 + 3.0, < 0.001) pancreatic insufficiency and 10 with pancreatic sufficiency, and to the control group. Similarly, the mean erythrocyte from 24 healthy controls. The mean maximal calcium-stimulated membrane (Na + K)ATPase activity was decreased only for specific activities, in the absence and presence of purified cal-those patients with a history of steatorrhea and who clinically Received August 18, 1983; accepted March 12, 1984. required pancreatic enzyme therapy and had low immunoreactive

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Erythrocyte Enigmas in Cystic Fibrosis

Cited by 9 publications

References 4 publications

Kinetic Parameters of Rubidium Transport Pathways Are Normal in Cystic Fibrosis Red Cells

Kinetic Parameters of Rubidium Transport Pathways Are Normal in Cystic Fibrosis Red Cells

Erythrocyte Transport Function in Cystic Fibrosis

Calcium-ATPase Activity in Cystic Fibrosis Erythrocyte Membranes: Decreased Activity in Patients with Pancreatic Insufficiency

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