Effects of N-acetylcysteine on dense cell formation in sickle cell disease

American Journal of Hematology

Volumn 73, Issue 1, 2003, Pages 26-32

  Pace, Betty S ^a   Shartava, Archil ^b   Pack Mabien, Ardie ^b   Mulekar, Mudhari ^b   Ardia, Alfredo ^c   Goodman, Steven R ^a  

^a UNIVERSITY OF TEXAS AT DALLAS   (United States)

^b UNIVERSITY OF SOUTH ALABAMA   (United States)

^c Zambon Corporation   (United States)

Author keywords

Dense cells; Glutathione; N acetylcysteine; Sickle cell disease; Vaso occlusive episodes

Indexed keywords

ACETYLCYSTEINE; GLUTATHIONE;

ADOLESCENT; ADULT; ARTICLE; CELL POPULATION; CELL TYPE; CLINICAL ARTICLE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DENSE CELL; DOUBLE BLIND PROCEDURE; DRUG EFFICACY; DRUG MECHANISM; DRUG SAFETY; DRUG TOLERABILITY; FEMALE; HUMAN; HUMAN CELL; MALE; OUTCOMES RESEARCH; OXIDATIVE STRESS; PERIPHERAL OCCLUSIVE ARTERY DISEASE; PHASE 2 CLINICAL TRIAL; PRIORITY JOURNAL; RANDOMIZED CONTROLLED TRIAL; SICKLE CELL; SICKLE CELL ANEMIA; SICKLE CELL CRISIS; VASOOCCLUSIVE DISEASE;

ACETYLCYSTEINE; ADOLESCENT; ADULT; ANEMIA, SICKLE CELL; DOUBLE-BLIND METHOD; ERYTHROCYTES, ABNORMAL; FEMALE; GLUTATHIONE; HUMANS; MALE; PLACEBOS; VASCULAR DISEASES;

EID: 0242600642     PISSN: 03618609     EISSN: None     Source Type: Journal    
DOI: 10.1002/ajh.10321     Document Type: Article

References (28)

1. Orringer EP, Abraham OJ, Parker JC. In: Embury SH, Hebbel RP, Mohandas N, Steinberg MH, editors. Sickle cell disease: basic principles and clinical practice. New York: Raven Press, Ltd.; 1994. p 861-871.
2. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995;332:1317-1322.
3. Wang WC, Wynn LW, Rogers ZR, Scott JP, Lane PA, Ware RE. A two year pilot trial of hydroxyurea in very young children with sickle cell anemia. J Pediatr 2001;139:790-796.
4. Gibson XA, Shartava A, McIntyre J, Monteiro CA, Zhang Y, Shah A, Campbell NF, Goodman SR. The efficacy of reducing agents or antioxidants in blocking the formation of dense cells and irreversibly sickled cells in vitro. Blood 1998;91:4373-4378.
5. Grassi C, Morandine C. A controlled trial of intermittent oral acetylcysteine in the long-term treatment of chronic bronchitis. Eur J Clin Pharmacol 1976;9:393-396.
6. Miller LF, Rumack BH. Clinical safety of high oral doses of acetylcysteine. Semin Oncol 1983;10:76-85.
7. Goodman SR. The role of the membrane skeleton in formation of the irreversibly sickled cell: a review. Cell Mol Biol Lett 1996;1:105-117.
8. Shartava A, Monteiro CA, Bencsath FA, Schneider K, Chait BT, Gussio R, Casoria-Scott LA, Shah AK, Heuerman CA, Goodman SR. A post-translational modification of beta-actin contributes to the slow dissociation of the spectrin-protein 4.1-actin complex of irreversibly sickled cells. J Cell Biol 1995;128:805-815.
9. Kafferlein HU, Angerer J. Determination of N-acetyl-S-(N-methylcarbamoyl)cysteine (AMCC) in the general population using gas chromatography-mass spectrometry. J Environ Mon 1999;1:465-469.
10. Shartava A, Miranda P, Williams KN, Shah A, Monteiro CA, Goodman SR. High density sickle cell erythrocyte core membrane skeletons demonstrate slow temperature dependent dissociation. Am J Hematol 1996;51:215-220.
11. Shartava A, Korn W, Shah AK, Goodman SR. Irreversibly sickled cell beta-actin: defective filament formation. Am J Hematol 1997;55:97-103.
12. Goodman SR, Pace BS, Shartava A. New therapeutic approaches to sickle cell disease: targeting RBC membrane oxidative damage. Cell Mol Biol Lett 1998;3:403-411.
13. Kaul DK, Fabry ME, Nagel RL. Vaso-occlusion by sickle cells: evidence for selective trapping of dense red cells. Blood 1986;68:1162-1169.
14. Kaul DK, Fabry ME, Nagel RL. Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: pathophysiological implications. Proc Natl Acad Sci U S A 1989;86:3356-3362.
15. Fabry ME, Fine E, Rajonayagam U, Factor SM, Gore J, Sylla M, Nagel RL. Demonstration of endothelial adhesion of sickle cells in vivo: a distinct role for deformable sickle cell discocytes. Blood 1992;79:1602-1611.
16. Ballas SK, Smith ED. Red blood cell changes during the evolution of the sickle cell painful crisis. Blood 1992;79:2154-2163.
17. Billett HH, Kin K, Fabry ME, Nagel RL. The percentage of dense cells does not predict the incidence of sickle cell painful crises. Blood 1986;68:301-308.
18. Lande WM, Andrews DL, Clark MR, Braham NV, Black DM, Embury SH, Mentzer WC. The incidence of painful crises in homozygous sickle cell disease: correlation with red cell deformability. Blood 1988;72:2056-2064.
19. Bernard GR, Wheeler AP, Arons MM, Morris PE, Paz HL, Russell JA, Wright PE. A trial of antioxidants N-acetylcysteine and procysteine in ARDS. The Antioxidant in ARDS Study Group. Chest 1997;112:164-170.
20. Boman AA. Comparison between different methods for detecting bronchial hyperreactivity: methods of study. Eur J Respir Dis 1983;64:405-413.
21. Brocard H, Charpin J, Germounty J. Multicenter, double-blind study of oral acetylcysteine vs. placebo. J Eur Respir Dis 1980;61:65-69.
22. Hanley JG, Fitzgerald MX. Meconium ileus equivalent in older patients with cystic fibrosis. Br Med J 1983;286:1411-1413.
23. Gavish D, Breslow L. Lipoprotein(a) reduction by N-acetylcysteine. J Lancet 1991;337:203-204.
24. De Rosa SC, Zaretsky MD, Dubs JG, Roederer M, Anderson M, Green A, Mitra D, Watanabe N, Nakamura H, Tjioe I, Deresinski SC, Moore WA, Ela SW, Parks D, Herzenberg LA, Herzenberg LA. N-Acetylcysteine replenishes glutathione in HIV infection. Eur J Clin Invest 2000;30:841-842.
25. Bencsath FA, Shartava A, Monteiro CA, Goodman SR. Identification of the disulfide-linked peptide in irreversibly sickled cell beta-actin. Biochemistry 1996;35:4403-4408.
26. Kakhniashvili DG, Chaudhary T, Zimmer WE, Bencsath FA, Jardine I, Goodman SR. Erythrocyte spectrin is an E2 ubiquitin conjugating enzyme. Biochemistry 2001;38:11630-11642.
27. Monteiro CA, Gibson X, Shartava A, Goodman SR. Preliminary characterization of a structural defect in homozygous sickle cell alpha spectrin demonstrated by a rabbit autoantibody. Am J Hematol 1998;58:200-205.
28. Sangerman J, Kakhaiashivilio DG, Brown A, Shartava A, Goodman SR. Spectrin ubiquitination and oxidative stress: potential roles in blood and neurological disorders. Cell Mol Biol Lett 2001;6:607-636.