Cl- Permeabilities in Red Blood Cells and Peripheral Blood Lymphocytes from Cystic Fibrosis and Control Subjects

Boucher, Richard C.; Ross, Dennis W.; Knowles, Michael R.; Gatzy, J. T.; Parker, John C.

doi:10.1203/00006450-198412000-00024

Cited by 6 publications

(2 citation statements)

References 18 publications

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“…Historically, most investigation has centered on the trafficking and function of CFTR and mutant CFTR in these epithelial structures. Boucher et al [1984] investigated the possibility that the CF chloride defect extended to the non‐epithelial, RBC. This investigation examined RBC chloride ion transport, and found no significant differences between control and phenotypic CF subjects.…”

Section: Discussionmentioning

confidence: 99%

Cystic fibrosis transmembrane conductance regulator in human and mouse red blood cell membranes and its interaction with ecto‐apyrase

Sterling

Shah²,

Kim³

et al. 2004

J of Cellular Biochemistry

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Elevated blood ATP and increased red blood cell (RBC) ATP transport is associated with cystic fibrosis (CF). In this report, we demonstrate the presence of the wild-type and the DeltaF508 mutant form of the CF transmembrane conductance regulator protein in RBC membranes and its putative interaction with ecto-apyrase, an ATP hydrolyzing enzyme also present in the RBC membrane. RBC membranes of control and DeltaF508 individuals and of wild-type and CF transmembrane conductance regulator-knockout mice were examined by immunoblot using several antibodies directed against different epitopes of this protein. These experiments indicated that human RBC membranes contain comparable amounts of the wild-type CF transmembrane conductance regulator protein and the DeltaF508 mutant form of the protein, respectively. CF transmembrane conductance regulator protein was also detected in wild-type mouse RBC membranes but not in the gene knockout mouse RBC membranes. Antibodies directed against ecto-apyrase co-immunoprecipitated CF transmembrane conductance regulator protein of human RBC membranes indicating a physical interaction between these two membrane proteins consistent with ATP transport and extracellular hydrolysis. We conclude that RBCs are a significant repository of CF transmembrane conductance regulator protein and should provide a novel system for evaluating its expression and function.

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Section: Discussionmentioning

confidence: 99%

Cystic fibrosis transmembrane conductance regulator in human and mouse red blood cell membranes and its interaction with ecto‐apyrase

Sterling

Shah²,

Kim³

et al. 2004

J of Cellular Biochemistry

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“…Boucher et al (26) have presented evidence that conductive anion (Cl-) permeability in CF RBC is also normal, indicating that the defect in C1 permeability or C1 channel regulation manifest in sweat duct and respiratory epithelium (2-6) is not present in RBC. It would appear, therefore, that the RBC is of limited use either as model system for the study of CF membrane transport abnormalities or as the source of phenotypic markers of abnormal ion transport in CF.…”

Section: Normalmentioning

confidence: 99%

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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Infection and Immunity to Pseudomonas

Sorensen¹,

Waller

Klinger

1990

Cystic Fibrosis

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Cl- Permeabilities in Red Blood Cells and Peripheral Blood Lymphocytes from Cystic Fibrosis and Control Subjects

Cited by 6 publications

References 18 publications

Cystic fibrosis transmembrane conductance regulator in human and mouse red blood cell membranes and its interaction with ecto‐apyrase

Cystic fibrosis transmembrane conductance regulator in human and mouse red blood cell membranes and its interaction with ecto‐apyrase

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

Infection and Immunity to Pseudomonas

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