Expression of HbC and HbS, but not HbA, results in activation of K-Cl cotransport activity in transgenic mouse red cells

Blood

Volumn 103, Issue 6, 2004, Pages 2384-2390

  Romero, Jose R ^b   Suzuka, Sandra M ^b   Nagel, Ronald L ^b   Fabry, Mary E ^a  

^a ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

ALKANOIC ACID; CHLORIDE; COTRANSPORTER; HEMOGLOBIN A; HEMOGLOBIN C; HEMOGLOBIN S; ISOTONIC SOLUTION; OKADAIC ACID; POTASSIUM;

ANIMAL CELL; ARTICLE; CHLORIDE TRANSPORT; CONTROLLED STUDY; ERYTHROCYTE; ERYTHROCYTE VOLUME; GENE INACTIVATION; HOMOZYGOTE; KNOCKOUT MOUSE; MEAN CORPUSCULAR HEMOGLOBIN; MOUSE; NONHUMAN; POTASSIUM TRANSPORT; PRIORITY JOURNAL; PROTEIN CONTENT; PROTEIN EXPRESSION; PROTEIN FUNCTION; QUALITATIVE ANALYSIS; QUANTITATIVE ANALYSIS; RETICULOCYTE; TRANSGENIC MOUSE;

EID: 1542343934     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood-2003-01-0237     Document Type: Article

References (50)

1. Brugnara C. Characteristics of the volume- and chloride-dependent K transport in human erythrocytes homozygous for hemoglobin C. J Membr Biol. 1989;111:69-81.
2. Canessa M. Red cell volume related ion transport systems in hemoglobinopathies. Hematol Oncol Clin North Am. 1991;5:495-516.
3. Brugnara C. Membrane transport of Na and K and cell dehydration in sickle erythrocytes. Experientia. 1993;49:100-109.
4. Ellory JC, Hall AC. Human red cell volume regulation in hypotonic media. Comp Biochem Physiol A. 1988;90:533-537.
5. --dependent K+ efflux is highly expressed in young human red cells containing normal hemoglobin or HbS. J Membr Biol. 1987;97:97-105.
6. Canessa M, Romero JR, Lawrence C, Nagel RL, Fabry ME. Rate of activation and deactivation of K:Cl cotransport by changes in cell volume in hemoglobin SS, CC and AA red cells. J Membr Biol. 1994;142:349-362.
7. Brugnara C, Van Ha T, Tosteson DC. Acid pH induces formation of dense cells in sickle erythrocytes. Blood. 1989;74:487-495.
8. Bookchin RM, Ortiz OE, Lew VL. Evidence for a direct reticulocyte origin of dense red cells in sickle cell anemia. J Clin Invest. 1991;87:113-124.
9. Fabry ME, Romero J, Buchanan ID, et al. Rapid increase in red blood cell density driven by K:Cl cotransport in a subset of sickle cell anemia reticulocytes and discocytes. Blood. 1991;78:217-225.
10. Murphy JR. Hemoglobin CC disease: rheological properties or erythrocytes and abnormalities in cell water. J Clin Invest. 1968;47:1483-1495.
11. Fabry ME, Kaul DK, Raventos C, et al. Some aspects of the pathophysiology of homozygous Hb CC erythrocytes. J Clin Invest. 1981;67:1284-1291.
12. Brugnara C, Kopin AS, Bunn HF, Tosteson DC. Regulation of cation content and cell volume in hemoglobin erythrocytes from patients with homozygous hemoglobin C disease. J Clin Invest. 1985;75:1608-1617.
13. Bunn HF, Noguchi CT, Hofrichter J, et al. Molecular and cellular pathogenesis of hemoglobin SC disease. Proc Natl Acad Sci U S A. 1982;79:7527-7531.
14. Fabry ME, Kaul DK, Raventos-Suarez C, Chang H, Nagel RL. SC erythrocytes have an abnormally high intracellular hemoglobin concentration. Pathophysiological consequences. J Clin Invest. 1982;70:1315-1319.
15. Klipstein FA, Ranney HM. Electrophoretic components of the hemoglobin of red cell membranes. J Clin Invest. 1960;39:1894-1899.
16. Shaklai N, Sharma VS, Ranney HM. Interaction of sickle cell hemoglobin with erythrocyte membranes. Proc Natl Acad Sci U S A. 1981;78:65-68.
17. Steck TL, Kant JA, Preparation of impermeable ghosts and inside-out vesicles from human erythrocyte membranes. Methods Enzymol. 1974;31:172-180.
18. Reiss GH, Ranney HM, Shaklai N. Association of hemoglobin C with erythrocyte ghosts. J Clin Invest. 1982;70:946-952.
19. Hirsch RE, Rybicki AC, Fataliev NA, et al. A potential determinant of enhanced crystallization of Hbc: spectroscopic and functional evidence of an alteration in the central cavity of oxyHbC. Br J Haematol. 1997;98:583-588.
20. Olivieri O, Vitoux D, Galacteros F, et al. Hemoglobin variants and activity of the (K+Cl-) cotransport system in human erythrocytes. Blood. 1992;79:793-797.
21. Brugnara C, Van Ha T, Tosteson DC. Properties of K+ transport in resealed human erythrocyte ghosts. Am J Physiol. 1988;255:C346-C356.
22. Nagel RL, Krishnamoorthy R, Fattoum S, et al. The erythrocyte effects of haemoglobin O(ARAB). Br J Haematol. 1999;107:516-521.
23. Gibson JS, Cossins AR, Ellory JC. Oxygen-sensitive membrane transporters in vertebrate red cells. J Exp Biol. 2000;203(pt 9):1395-1407.
24. Greaves DR, Fraser P, Vidal MA, et al. A transgenic mouse model of sickle cell disorder. Nature. 1990;343:183-185.
25. Ryan TM, Townes TM, Reilly MP, et al. Human sickle hemoglobin in transgenic mice. Science. 1990;247:566-568.
26. Rubin EM, Witkowska HE, Spangler E, et al. Hypoxia-induced in vivo sickling of transgenic mouse red cells. J Clin Invest. 1991;87:639-647.
27. Trudel M, Saadane N, Garel M-C, et al. Towards a transgenic mouse model of sickle cell disease: hemoglobin SAD. EMBO J. 1991;10:3157-3168.
28. Fabry ME, Costantini F, Pachnis A, et al. High expression of human β S- and α-globins in transgenic mice: erythrocyte abnormalities, organ damage, and the effect of hypoxia. Proc Natl Acad Sci U S A. 1992;89:12155-12159.
29. Romero JR, Fabry ME, Suzuka SM, et al. K:Cl cotransport in red cells of transgenic mice expressing high levels of human hemoglobin S. Am J Hematol. 1997;55:112-114.
30. Armsby CC, Brugnara C, Alper SL. Cation transport in mouse erythrocytes: role of K(+)-Cl-cotransport in regulatory volume decrease. Am J Physiol. 1995;268:(pt 1):C894-C902.
31. Fabry ME, Romero JR, Suzuka SM, et al. Hemoglobin C in transgenic mice: effect of HbC expression from founders to full mouse globin knockouts. Blood Cells Mol Dis. 2000;26:331-347.
32. Fabry ME, Suzuka SM, Weinberg RS, et al. Second generation knockout sickle mice: the effect of HbF. Blood. 2001;97:410-418.
33. Romero JR, Suzuka SM, Nagel RL, Fabry ME. Arginine supplementation of sickle transgenic mice reduces red cell density and Gardos channel activity. Blood. 2002;99:1103-1108.
34. Fabry ME, Nagel RL, Pachnis A, Suzuka SM, Costantini F. High expression of human β S- and α-globins in transgenic mice: hemoglobin composition and hematological consequences. Proc Natl Acad Sci U S A. 1992;89:12150-12154.
35. Aγ (abstract]. Blood. 1995;86:648a.
36. Paszty C, Mohandas N, Stevens ME, et al. Lethal alpha-thalassaemia created by gene targeting in mice and its genetic rescue. Nat Genet. 1995;11:33-39.
37. Yang B, Kirby S, Lewis J, et al. A mouse model for β 0-thalassemia. Proc Natl Acad Sci U S A. 1995;92:11608-11612.
38. Ryan TM, Ciavatta DJ, Townes TM. Knockout-transgenic mouse model of sickle cell disease. Science. 1997;278:873-876.
39. Paszty C, Brion CM, Manci E, et al. Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease [see comments]. Science. 1997;278:876-878.
40. Fabry ME, Mears JG, Patel P, et al. Dense cells in sickle cell anemia: the effects of gene interaction. Blood. 1984;64:1042-1046.
41. Ciavatta DJ, Ryan TM, Farmer SC, Townes TM. Mouse model of human beta zero thalassemia: targeted deletion of the mouse beta maj- and beta min-globin genes in embryonic stem cells. Proc Natl Acad Sci U S A. 1995;92:9259-9263.
42. Skow LC, Burkhart BA, Johnson FM, et al. A mouse model for beta-thalassemia. Cell. 1983;34:1043-1052.
43. Hall AC, Ellory JC. Evidence for the presence of volume-sensitive KCl transport in 'young' human red cells. Biochim Biophys Acta. 1986;858:317-320.
44. Johnstone RM. The Jeanne Manery-Fisher Memorial Lecture 1991. Maturation of reticulocytes: formation of exosomes as a mechanism for shedding membrane proteins. Biochem Cell Biol. 1992;70:179-190.
45. Jennings ML, Schulz RK. Okadaic acid inhibition of KCl cotransport. Evidence that protein dephosphorylation is necessary for activation of transport by either cell swelling or N-ethylmaleimide. J Gen Physiol. 1991;97:799-817.
46. Vitoux D, Olivieri O, Garay RP, et al. Inhibition of K+ efflux and dehydration of sickle cells by [(di-hydroindenyl)oxy]alkanoic acid: an inhibitor of the K+ Cl-cotransport system. Proc Natl Acad Sci U S A. 1989;86:4273-4276.
47. Delpire E, Mount DB. Human and murine phenotypes associated with defects in cation-chloride cotransport. Annu Rev Physiol. 2002;64:803-843.
48. Mercado A, Song L, Vazquez N, Mount DB, Gamba G. Functional comparison of the K+-Cl-cotransporters KCC1 and KCC4. J Biol Chem. 2000;275:30326-30334.
49. Race JE, Makhlouf FN, Logue PJ, et al. Molecular cloning and functional characterization of KCC3, a new K-Cl cotransporter. Am J Physiol. 1999;277:C1210-C1219.
50. Lauf PK, Zhang J, Delpire E, et al. K-Cl co-transport: immunocytochemical and functional evidence for more than one KCC isoform in high K and low K sheep erythrocytes. Comp Biochem Physiol A Mol Integr Physiol. 2001;130:499-509.