A transgenic mouse model of hemoglobin S Antilles disease

Blood

Volumn 89, Issue 11, 1997, Pages 4204-4212

  Popp, R A ^a   Popp, D M ^b   Shinpock, S G ^b   Yang, M Y ^b   Mural, J G ^b   Aguinaga, M D P ^b   Kopsombut, P ^b   Roa, P D ^b   Turner, E A ^b   Rubin, E M ^b  

^a OAK RIDGE NATIONAL LABORATORY   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

HEMOGLOBIN S;

AMINO ACID SUBSTITUTION; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; ERYTHROCYTE; GENE CONSTRUCT; MOUSE; NONHUMAN; OXYGEN AFFINITY; PHENOTYPE; PRIORITY JOURNAL; PROTEIN POLYMERIZATION; RETICULOCYTOSIS; SCANNING ELECTRON MICROSCOPY; SICKLE CELL ANEMIA; TRANSGENE; TRANSGENIC MOUSE;

EID: 0030905710     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood.v89.11.4204     Document Type: Article

References (23)

1. Monplaisir N, Merault G, Poyart C, Rhoda M-D, Craescu C, Vidaud M, Galacteros F, Blouquit Y, Rosa J: Hemoglobin S Antilles: A variant with lower solubility than hemoglobin S and producing sickle cell disease in heterozygotes. Proc Natl Acad Sci USA 83:9363, 1986
2. Greaves RG, Frazer P, Vidal MA, Hedges MJ, Roper D, Luzzatto L, Grosveld F: A transgenic mouse model for sickle cell disorder. Nature 343:183, 1990
3. Ryan TM, Townes TM, Reilly MP, Asakura T, Palmiter RD, Brinster RL, Behringer RR: Human sickle hemoglobin in transgenic mice. Science 247:566, 1990
4. Rubin EM, Witkowska HE, Spangler E, Curtin R, Lubin BH, Mohandas N, Clift SM: Hypoxia-induced in vivo sickling of transgenic mouse red cells. J Clin Invest 87:639, 1991
5. s-and α-globins in transgenic mice: Erythrocyte abnormalities, organ damage, and the effect of hypoxia. Proc Natl Acad Sci USA 89:12155, 1992
6. Fabry ME, Sengupta A, Suzuka SM, Costantini F, Rubin EM, Hofrichter J, Christoph G, Manci E, Culberson D, Factor SM, Nagel RL: A second transgenic mouse model expressing both hemoglobin S (HbS) and HbS-Antilles results in increased phenotypic severity. Blood 86:2419, 1995
7. Popp RA, Marsh CL, Skow LC: Expression of embryonic hemoglobin genes in mice heterozygous for α-thalassemia or β-duplication traits and in mice heterozygous for both traits. Develop Biol 85:123, 1981
8. Bunn HF, Forget BG: Hemoglobin: Molecular, Genetic and Clinical Aspects. Philadelphia, PA, Saunders, 1986
9. D'Surney SJ, Popp RA: Oxygen association-dissociation and stability analysis on mouse hemoglobins with mutant alpha and beta globins. Genetics 132:545, 1992
10. Popp RA, Shinpock SG, Popp DM, Russell KD, Poole TL, Rubin EM: In vivo sickling of red cells and pathobiology in a newly developed transgenic HbS Antilles line of mice. Natl Sickle Cell Dis Prog 18:3a, 1993 (abstr)
11. Popp DM, Popp RA, Lock S, Mann RC, Hand JR: The use of multiparameter analysis to quantitate hematological damage from exposure to a chemical (ethylene oxide). J Toxicol Environ Health 18:543, 1986
12. Wintrobe MM, Lee GR, Boggs DR, Bithell TC, Forester J, Athens JW, Lukens JN (eds): Clinical Hematology (ed 8). Philadelphia. PA, Lea and Febiger, 1981
13. Davis BH, Bigelow N, Ball ED, Mills L, Cornwell J: Utility of flow cytometric reticulocyte quantification as a predictor of engraftment in autologous bone marrow transplantation. Am J Hematol 32:81, 1989
14. Gutierrez C, Bernabe RR, Vega J, Kreisler M: Purification of human T and B cells by a discontinuous density gradient of Percoll. J Immunol Methods 29:57, 1979
15. Festa RS, Akasura T: The use of an oxygen dissociation curve analyser in transfusion therapy. Transfusion 19:107, 1979
16. Roa PD, Turner EA, Aguinaga MdP: Reference ranges for hemoglobin variants by HPLC in African Americans. Ann Clin Lab Sci 25:228, 1995
17. Aγ ratio in fetal haemoglobin and to studies of globin synthesis. Br J Haematol 44:527, 1980
18. Reilly MP, Chomo MJ, Obata K, Brener R, Asakura T: Pathological changes seen in transgenic mice which produce human sickle hemoglobin under normoxic and hypoxic conditions. Blood 80:78a, 1992 (abstr, suppl 1)
19. Trudel M, Saadane N, Garel M-C, Bardakdjian-Michau J, Blouquit Y, Guerquin-Kern J-L, Rouyer-Fessard P, Vidaud D, Pachnis A, Romeo P-H, Beuzard Y, Costantini F: Towards a transgenic mouse model of sickle cell disease: Hemoglobin SAD. EMBO J 10:3157, 1991
20. Trudel M, De Paepe ME, Chretien N, Saadane N, Jacmain J, Sorette M, Hoang T, Beuzard Y: Sickle cell disease of transgenic SAD mice. Blood 84:3189, 1994
21. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR, and the Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 332:1317, 1995
22. Mulligan RC: The basic science of gene therapy. Science 260:926, 1993
23. Walters MC, Patience M, Leisenring W, Eckman JR, Scott JP, Mentzer WC, Davies SC, Ohene-Frempong K, Bernaudin F, Matthews DC, Storb R, Sullivan KM: Bone marrow transplantation for sickle cell disease. N Engl J Med 335:369, 1996