Transgenic mice overexpressing the human Cu/Zn-SOD gene: Ultrastructural studies of a premature thymic involution model of Down's syndrome (trisomy 21)

Laboratory Investigation

Volumn 74, Issue 3, 1996, Pages 617-626

  Nabarra, Bernadette ^a   Casanova, Myriam ^a   Paris, Daniel ^a,b   Nicole, Annie ^a   Toyama, Kyoko ^a   Sinet, Pierre Marie ^a   Ceballos, Isabelle ^a   London, Jacqueline ^a  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

^b UNIVERSITÉ PARIS DESCARTES   (France)

Author keywords

[No Author keywords available]

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE;

ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN; CHROMOSOME 21; CONTROLLED STUDY; DOWN SYNDROME; ELECTRON MICROSCOPY; ENZYME ACTIVITY; GENE; GENE EXPRESSION; MOUSE; NONHUMAN; PHENOTYPE; POLYMERASE CHAIN REACTION; PRIORITY JOURNAL; SOUTHERN BLOTTING; SPLEEN; THYMUS; TRANSGENIC MOUSE; ULTRASTRUCTURE;

ANIMALS; DISEASE MODELS, ANIMAL; DOWN SYNDROME; FEMALE; GENE EXPRESSION REGULATION, ENZYMOLOGIC; HUMANS; MALE; MICE; MICE, INBRED BALB C; MICE, INBRED DBA; MICE, TRANSGENIC; MICROSCOPY, ELECTRON; SUPEROXIDE DISMUTASE; THYMUS GLAND;

EID: 0029983348     PISSN: 00236837     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (57)

1. Aita M and Amantea A (1991). Distribution of anti-keratines and anti-thymostimuline antibodies in normal and in Down's syndrome human thymus. Thymus 17:155-159.
2. Avraham K, Schickler M, Sapoznikow R, Yarom R, and Groner Y (1988). Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with levels of human Cu/Zn superoxide dismutase. Cell 54:823-829.
3. Avraham K, Sugaman H, Rotshenker S, and Groner Y (1991). Down's syndrome: Morphological remodelling and increased complexity in the neuromuscular junction of transgenic CuZn-superoxide dismutase mice. J Neurocytol 20:208-215.
4. Baribault H, Penner J, Lozzo R, and Wilson-Heinez M (1994). Colorectal hyperplasia and imflammation in keratin 8 deficient FVB/N mice. Gene Dev 8:2964-2973.
5. Barkats M, Bertholet JY, Vanault P, Ceballos-Picot I, Nicole A, Phillips J, Moutier R, Roubertoux P, Sinet PM, and Cohen-Salmon C (1993). Hippocampal mossy fiber changes in transgenic mice for the human copper-zinc superoxide dismutase. Neurosci Lett 160:24-28.
6. Benda CE and Strassman GS (1965). The thymus in mongolism. J Ment Defic Res 9:109-117.
7. Bertotto A, Gerli R, Fabietti G, Arcangeli CDF, Bianchi S, and Vaccaro R (1984). Immunodeficiency in Down's syndrome. Analysis by OKT monoclonal antibodies. Thymus 6:365-367.
8. Bertotto A, Scalise F, Gerli R, and Castelucci G (1992). Lymphocytes bearing the γ/δ T-cell receptors in Down's syndrome. Scand J Immunol 35:275-278.
9. Burgio GR, Ugazio A, Nespoli L, and Maccario R (1983). Down's syndrome: A model of immunodeficiency. Birth Defects 19:325-327.
10. Ceballos-Picot I, Nicole A, Briand P, Grimber G, and Delacourte A (1991a). Neuronal specific expression of human copper-zinc superoxide dismutase gene in transgenic mice: Animal model of gene dosage effects in Down's syndrome. Brain Res 552:198-214.
11. Ceballos-Picot I, Nicole A, Briand P, Grimber G, Delacourte A, Flament S, Brown JL, Thevenin M, Kamoun P, and Sinet PM (1991b). Expression of human Cu/Zn superoxide dismutase gene in transgenic mice: Model for gene dosage effect in Down's syndrome. Free Radical Res Commun 12:581-589.
12. Ceballos-Picot I, Nicole A, Clement M, Bourre JM, and Sinet PM (1992). Age-related changes in antioxidant enzymes and lipid peroxidation in brains of control and transgenic mice overexpressing copper-zinc superoxide dismutase. Mutat Res 275:281-293.
13. Chan PH, Yang GY, Chen SF, Carson E, and Epstein CJ (1990). Cold induced brain aedema and infarction are reduced in transgenic mice overexpressing CuZn superoxide dismutase. Ann Neurol 29:482-486.
14. Clarke A (1986). The many facets of thymic involution. Immunol Today 7:204-207.
15. Epstein CJ, Avraham KB, Lovett M, Smith S, Elroy-Stein O, Rotman G, Bry C, and Groner Y (1987). Transgenic mice with increased CuZn-superoxyde dismutase activity: An animal model of dosage effects in Down's syndrome. Proc Natl Acad Sci USA 84:8044-8048.
16. Fonseca E, Lannes-Viera J, Villa-Verde D, Savino W (1989). Thymic extracellular matrix in DS. Bras J Med Biol Res 22:971-974.
17. Fransceshi C, Licastro F, Chiricolo M, Boetti F, Zannotti M, Fabris N, Mochegiani E, Fantanini MP, Paolucci P, and Masi M (1981). Deficiency of autologous mixed lymphocytes reactions and serum thymic factor level in Down's syndrome. J Immunol 126:2161-2164.
18. Fridovich I (1986). Superoxidase dismutases. In: Meister A, editor. Advances in enzymology. New York: John. Wiley and Sons, 61.
19. Gowan W and Sorenson G (1964). Electron microscopic observations of acute thymic involution produced by hydrocortisone. Lab Invest 13:353-356.
20. Groner Y, Elroy-Stein O, Bernstein Y, Dafni N, Levanon D, Danciger E, and Neer A (1996). Molecular expression of Down's syndrome: Overexpression of transfected TSVP human CUZN-superoxide dismutase gene and the consequent physiological changes. Cold Spring Harbor Symp Quant Biol 51:381-393.
21. Halliwell B and Gutteridge JM (1985). The importance of free radicals and catalytic metal ions in human diseases. Mol Aspects Med 8:89-193.
22. Hallewell RA, Puma JP, Mullenbach G, and Najarian R (1986). Structure of the human Cu/Zn-SOD gene. In: Rotilio, editor. Superoxide dismutase in chemistry, biology and medicine. Amsterdam: Elsevier Science, 249.
23. Hirokawa K (1977). The thymus and aging. In: Makinodan T and Yunis E, editors. Immunology and aging. New York: Plenum Press, 51.
24. Kay M and Makinodan T (1981). Relationship between aging and the immune system. Prog Allergy 29:134-181.
25. Kyewski B and Rüther U (1989). Thymus histopathology in mice transgenic for c-fos and Ha-Ras. Thymus 14:89-93.
26. Larocca LM, Piatelli M, Valitutti S, Castellino F, Maggiano N, and Musiani P (1988). Alterations in thymocyte subpopulations in Down's syndrome (trisomy 21). Clin Immunol Immunopathol 49:175-186.
27. Levin S (1987). The immune system and susceptibility to infection in Down's syndrome. In: McCoy E and Epstein I, editors. Oncology and immunology of Down's syndrome. New York: AR Liss, 143-146.
28. Levin S, Schlesinger M, Handzel Z, Hahn T, Altman Y, Czernoblisky B, and Boss J (1979). Thymic deficiency in Down's syndrome. Pediatrics 63:80-87.
29. Liss L, Shim C, and Thase M (1980). The relationship between Down's syndrome and dementia Alzheimer's type. J Neuropathol Exp Neurol 39:371-375.
30. Manikodan T (1977). Immunity and aging. In: Finck C and Hayflick L, editors. Handbook of the biology of aging. New York: Van Nostrand-Reinhold, 379-408.
31. Marrack P and Kappler J (1987). The T cell receptor. Science 238:1073-1079.
32. Moll J, Eibel H, and Schmid P (1992). Thymic hyperplasia in transgenic mice caused by immortal epithelial cells expressing c-kit ligand. Eur J Immunol 22:1587-1594.
33. Murphy M and Epstein LB (1990). Down's syndrome (Trisomy 21) thymuses have a decreased proportion of cells expressing high levels of TCR αβ and CD3. Clin Immunol Immunopathol 55:453-467.
34. Murphy M and Epstein LB (1992). Down's syndrome (DS) peripheral blood: Evidence for an inefficient release of mature T cells by the DS thymus. Immunol Immunopathol 62:245-251.
35. Murphy M, Friend DS, Pike-Nobile L, and Epstein LB (1992). Tumor necrosis-α factor and IFN-τ expression in human thymus: Localization and overexpression in Down's syndrome (Trisomy 21). J Immunol 149:2506-2512.
36. Nabarra B and Andrianarison I (1987). Ultrastructural studies of thymic reticulum. I. Epithelial components. Thymus 9:95-100.
37. Nabarra B and Andrianarison I (1991). Ultrastructural studies of thymic reticulum. II. Non-epithelial components. Thymus 17:39-43.
38. Nabarra B and Andrianarison I (1995). Ultrastructural studies of thymic reticulum. III. Connective components. Tissue Cells 27:249-261.
39. Nabarra B and Andrianarison I (in press, 1996). Ultrastructural study of thymic microenvironment involution in aging mice. Exp Gerontol.
40. Nabarra B and Papiernik M (1988). Phenotype of thymic stromal cells. An immunoelectron microscopy study. Lab Invest 48:524-527.
41. Noble R and Warren R (1987). Altered T-cell subsets and defective T-cell function in young children with Down's syndrome. Immunol Invest 16:371-382.
42. Philip R, Berger A, McManus N, Warner N, Peacock M, and Epstein LB (1986). Abnormalities of the in vitro cellular and humoral responses to tetanus and influenza antigens with concomitant numerical alterations in lymphocyte subsets in Down's syndrome. J Immunol 136:1661-1667.
43. Przedborski S, Jackson-Lewis V, Kostic V, Carlson E, Epstein CJ, and Cadet JL (1992a) Superoxide dismutase, catase and glutathione peroxidase activities in copper/zinc-superoxide dismutase transgenic mice. J Neurochem 58:1760-1767.
44. Przedborski S, Kostic V, Jackson-Lewis V, Naini AB, Simonetti S, Fahn S, Carlson E, Epstein CJ, and Cadet JL (1992b) Transgenic mice with increased CuZn superoxide dismutase activity are resistant to N-methyl-4-phenyl-1,2,3,6,-tetrahydropyridine induced neurotoxicity. J Neurosci 12: 1658-1667.
45. Pueschel S (1990). Clinical aspects of Down's syndrome from infancy to adulthood. Am J Med Genet 7(Suppl):52-56.
46. Rüther U, Müller W, and Sumida T (1988). c-Fos expression interferes with thymus development in transgenic mice. Cell 53:847-856.
47. Schickler, M, Knobler H, Avraham KB, Elroy-Stein O, and Groner Y (1989). Diminished serotonin uptake in platelets of transgenic mice with increased Cu/Zn-superoxide dismutase activity. EMBO J 8:1385-1392.
48. Schuurman H, Nagelkerken L, de Weger R, and Rozing J (1992). Age associated involution: Significance of a physiological process. Thymus 18:1-56.
49. Shen M, Radek C, and Cardiff R (1994). Expression of LIF in transgenic mice results in altered thymic epithelium and apparent interconversion of thymic and lymph node morphologies. EMBO J 13:1375-1385.
50. Simson J, Wang J, Chao J, and Chao L (1994). Histopathology of lymphatic tissues in transgenic mice expressing human tissue kallikrein gene. Lab Invest 71:680-687.
51. Sinet PM (1982). Metabolism of oxygen derivatives in Down's syndrome. Ann N Y Acad Sci 396:83-94.
52. 2. Arch Biochem Biophys 212:411-416.
53. Van Ewijk W (1991). T-cell differentiation is influenced by thymic microenvironments. Annu Rev Immunol 9:591-615.
54. Van Ewijk W, Kisielow P, and von Boehmer H (1990). Immunohistology of T cell differentiation in the thymus of H-Y specific T cell receptor α/β transgenic mice. Eur J Immunol 20:129-137.
55. Von Boehmer H (1988). The developmental biology of T lymphocytes. Annu Rev Immunol 6:306-326.
56. Walford R, Gesset T, Marim F, and Tam C (1981). Immunological and biochemical studies of Down's syndrome as a model of accelerated aging. In: Segre D and Smith L, editors. Immunological aspects of aging. New York: Marcel Dekker, 479.
57. Yim MB, Chock PB, and Stadman ER (1990). Copper-zinc superoxide dismutase catalyses hydroxyl radicals production from hydrogene peroxide. Proc Natl Acad Sci USA 87:5006-5010.