Improved muscle strength and mobility in the dy2J/dy2J mouse with merosin deficient congenital muscular dystrophy treated with Glatiramer acetate

Neuromuscular Disorders

Volumn 20, Issue 4, 2010, Pages 267-272

  Dadush, Oshrat ^a   Aga Mizrachi, Shlomit ^a   Ettinger, Keren ^a   Tabakman, Rinat ^a   Elbaz, Moran ^a   Fellig, Yakov ^a   Yanay, Nurit ^a   Nevo, Yoram ^a  

^a HADASSAH HEBREW UNIVERSITY MEDICAL CENTER   (Israel)

Author keywords

Anti inflammatory agent; Congenital muscular dystrophy; Dy2J dy2J mice; Fibrosis; Glatiramer acetate; Regeneration

Indexed keywords

FIBRONECTIN; GLATIRAMER; LAMININ ALPHA2; MYOD PROTEIN; MYOGENIN; VIMENTIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; AUTOSOMAL RECESSIVE DISORDER; C57BL 6 MOUSE; CONTROLLED STUDY; DISEASE MODEL; DRUG EFFICACY; FEMALE; GENE EXPRESSION; GRIP STRENGTH; HINDLIMB; MALE; MEROSIN DEFICIENT CONGENITAL MUSCULAR DYSTROPHY; MOTOR PERFORMANCE; MOUSE; MUSCLE STRENGTH; MUSCULAR DYSTROPHY; NONHUMAN; PRIORITY JOURNAL;

ANIMALS; DISEASE MODELS, ANIMAL; FEMALE; FIBRONECTINS; FIBROSIS; HINDLIMB; IMMUNOSUPPRESSIVE AGENTS; LAMININ; MALE; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MOTOR ACTIVITY; MUSCLE DEVELOPMENT; MUSCLE STRENGTH; MUSCLE WEAKNESS; MUSCLE, SKELETAL; MUSCULAR DYSTROPHY, ANIMAL; MYOD PROTEIN; MYOGENIN; PEPTIDES; REGENERATION; VIMENTIN;

EID: 77950954615     PISSN: 09608966     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.nmd.2010.02.002     Document Type: Article

References (59)

1. Lisi M.T., Cohn R.D. Congenital muscular dystrophies: new aspects of an expanding group of disorders. Biochim Biophys Acta 2007, 1772:159-172.
2. Miyagoe-Suzuki Y., Nakagawa M., Takeda S. Merosin and congenital muscular dystrophy. Microsc Res Tech 2000, 48:181-191.
3. Muntoni F., Voit T. The congenital muscular dystrophies in 2004: a century of exciting progress. Neuromuscul Disord 2004, 14:635-649.
4. Jimenez-Mallebrera C., Brown S.C., Sewry C.A., Muntoni F. Congenital muscular dystrophy: molecular and cellular aspects. Cell Mol Life Sci 2005, 62:809-823.
5. Talim B., Kale G., Topaloglu H., et al. Clinical and histopathological study of merosin-deficient and merosin-positive congenital muscular dystrophy. Pediatr Dev Pathol 2000, 3:168-176.
6. Xu H., Wu X.R., Wewer U.M., Engvall E. Murine muscular dystrophy caused by a mutation in the laminin alpha 2 (Lama2) gene. Nat Genet 1994, 8:297-302.
7. Vilquin J.T., Vignier N., Tremblay J.P., Engvall E., Schwartz K., Fiszman M. Identification of homozygous and heterozygous dy2J mice by PCR. Neuromuscul Disord 2000, 10:59-62.
8. Colognato H., Yurchenco P.D. The laminin alpha2 expressed by dystrophic dy(2J) mice is defective in its ability to form polymers. Curr Biol 1999, 9:1327-1330.
9. Vainzof M., Ayub-Guerrieri D., Onofre P.C., et al. Animal models for genetic neuromuscular diseases. J Mol Neurosci 2008, 34:241-248.
10. Shorer Z., Philpot J., Muntoni F., Sewry C., Dubowitz V. Demyelinating peripheral neuropathy in merosin-deficient congenital muscular dystrophy. J Child Neurol 1995, 10:472-475.
11. Schessl J., Zou Y., Bonnemann C.G. Congenital muscular dystrophies and the extracellular matrix. Semin Pediatr Neurol 2006, 13:80-89.
12. Spencer M.J., Tidball J.G. Do immune cells promote the pathology of dystrophin-deficient myopathies?. Neuromuscul Disord 2001, 11:556-564.
13. Morrison J., Palmer D.B., Cobbold S., Partridge T., Bou-Gharios G. Effects of T-lymphocyte depletion on muscle fibrosis in the mdx mouse. Am J Pathol 2005, 166:1701-1710.
14. Tidball J.G. Inflammatory processes in muscle injury and repair. Am J Physiol Regul Integr Comp Physiol 2005, 288:R345-R353.
15. Farini A., Meregalli M., Belicchi M., et al. T and B lymphocyte depletion has a marked effect on the fibrosis of dystrophic skeletal muscles in the scid/mdx mouse. J Pathol 2007, 213:229-238.
16. Vidal B., Serrano A.L., Tjwa M., et al. Fibrinogen drives dystrophic muscle fibrosis via a TGF-β/alternative macrophage activation pathway. Genes Dev 2008, 22:1747-1752.
17. Butterfield T.A., Best T.M., Merrick M.A. The dual roles of neutrophils and macrophages in inflammation: a critical balance between tissue damage and repair. J Athl Train 2006, 41:457-465.
18. Cohn R.D., van Erp C., Habashi J.P., et al. Angiotensin II type 1 receptor blockade attenuates TGF-β-induced failure of muscle regeneration in multiple myopathic states. Nat Med 2007, 13:204-210.
19. Andreetta F., Bernasconi P., Baggi F., et al. Immunomodulation of TGF-β 1 in mdx mouse inhibits connective tissue proliferation in diaphragm but increases inflammatory response: implications for antifibrotic therapy. J Neuroimmunol 2006, 175:77-86.
20. Gosselin L.E., Barkley J.E., Spencer M.J., McCormick K.M., Farkas G.A. Ventilatory dysfunction in mdx mice. impact of tumor necrosis factor-alpha deletion. Muscle Nerve 2003, 28:336-343.
21. Hodgetts S., Radley H., Davies M., Grounds M.D. Reduced necrosis of dystrophic muscle by depletion of host neutrophils, or blocking TNFalpha function with Etanercept in mdx mice. Neuromuscul Disord 2006, 16:591-602.
22. Arnon R., Aharoni R. Mechanism of action of Glatiramer acetate in multiple sclerosis and its potential for the development of new applications. Proc Natl Acad Sci USA 2004, 101(SUPPL. 2):14593-14598.
23. Fridkis-Hareli M., Teitelbaum D., Gurevich E., et al. Direct binding of myelin basic protein and synthetic copolymer 1 to class II major histocompatibility complex molecules on living antigen-presenting cells-specificity and promiscuity. Proc Natl Acad Sci USA 1994, 91:4872-4876.
24. Vieira P.L., Heystek H.C., Wormmeester J., Wierenga E.A., Kapsenberg M.L. Glatiramer acetate (copolymer-1, copaxone) promotes Th2 cell development and increased IL-10 production through modulation of dendritic cells. J Immunol 2003, 170:4483-4488.
25. Neuhaus O., Farina C., Yassouridis A., et al. Multiple sclerosis: comparison of copolymer-1- reactive T cell lines from treated and untreated subjects reveals cytokine shift from T helper 1 to T helper 2 cells. Proc Natl Acad Sci USA 2000, 97:7452-7457.
26. Duda P.W., Schmied M.C., Cook S.L., Krieger J.I., Hafler D.A. Glatiramer acetate (Copaxone) induces degenerate, Th2-polarized immune responses in patients with multiple sclerosis. J Clin Invest 2000, 105:967-976.
27. Tanase K., Teng Q., Krishnaney A.A., Liu J.K., Garrity-Moses M.E., Boulis N.M. Cervical spinal cord delivery of a rabies G protein pseudotyped lentiviral vector in the SOD-1 transgenic mouse. Invited submission from the Joint Section Meeting on Disorders of the Spine and Peripheral Nerves, March 2004. J Neurosurg Spine 2004, 1:128-136.
28. Boguszewski P., Zagrodzka J. Emotional changes related to age in rats-a behavioral analysis. Behav Brain Res 2002, 133:323-332.
29. de Souza Silva M.A., Topic B., Huston J.P., Mattern C. Intranasal dopamine application increases dopaminergic activity in the neostriatum and nucleus accumbens and enhances motor activity in the open field. Synapse 2008, 62:176-184.
30. Pan Y., Chen C., Shen Y., et al. Curcumin alleviates dystrophic muscle pathology in mdx mice. Mol Cells 2008, 25:531-537.
31. Guo L.T., Zhang X.U., Kuang W., et al. Laminin alpha2 deficiency and muscular dystrophy; genotype-phenotype correlation in mutant mice. Neuromuscul Disord 2003, 13:207-215.
32. Morrison J., Lu Q.L., Pastoret C., Partridge T., Bou-Gharios G. T-cell-dependent fibrosis in the mdx dystrophic mouse. Lab Invest 2000, 80:881-891.
33. Wehling M., Spencer M.J., Tidball J.G. A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice. J Cell Biol 2001, 155:123-131.
34. Miller A., Shapiro S., Gershtein R., et al. Treatment of multiple sclerosis with copolymer-1 (Copaxone): implicating mechanisms of Th1 to Th2/Th3 immune-deviation. J Neuroimmunol 1998, 92:113-121.
35. Aharoni R., Kayhan B., Eilam R., Sela M., Arnon R. Glatiramer acetate-specific T cells in the brain express T helper 2/3 cytokines and brain-derived neurotrophic factor in situ. Proc Natl Acad Sci USA 2003, 100:14157-14162.
36. Aharoni R., Aizman E., Fuchs O., Arnon R., Yaffe D., Sarig R. Transplanted myogenic progenitor cells express neuronal markers in the CNS and ameliorate disease in experimental autoimmune encephalomyelitis. J Neuroimmunol 2009, 215:73-83.
37. Ruggieri M., Avolio C., Livrea P., Trojano M. Glatiramer acetate in multiple sclerosis: a review. CNS Drug Rev 2007, 13:178-191.
38. Aharoni R., Teitelbaum D., Sela M., Arnon R. Bystander suppression of experimental autoimmune encephalomyelitis by T cell lines and clones of the Th2 type induced by copolymer 1. J Neuroimmunol 1998, 91:135-146.
39. Blanchette F., Neuhaus O. Glatiramer acetate: evidence for a dual mechanism of action. J Neurol 2008, 255(SUPPL. 1):26-36.
40. Aharoni R., Yussim A., Sela M., Arnon R. Combined treatment of Glatiramer acetate and low doses of immunosuppressive drugs is effective in the prevention of graft rejection. Int Immunopharmacol 2005, 5:23-32.
41. Horani A., Muhanna N., Pappo O., et al. Beneficial effect of Glatiramer acetate (Copaxone) on immune modulation of experimental hepatic fibrosis. Am J Physiol Gastrointest Liver Physiol 2007, 292:G628-G638.
42. Kadler K.E., Hill A., Canty-Laird E.G. Collagen fibrillogenesis: fibronectin, integrins, and minor collagens as organizers and nucleators. Curr Opin Cell Biol 2008, 20:495-501.
43. Dallas S.L., Chen Q., Sivakumar P. Dynamics of assembly and reorganization of extracellular matrix proteins. Curr Top Dev Biol 2006, 75:1-24.
44. Mezzano V., Cabrera D., Vial C., Brandan E. Constitutively activated dystrophic muscle fibroblasts show a paradoxical response to TGF-β and CTGF/CCN2. J Cell Commun Signal 2007, 1:205-217.
45. Cizkova D., Soukup T., Mokry J. Expression of nestin, desmin and vimentin in intact and regenerating muscle spindles of rat hind limb skeletal muscles. Histochem Cell Biol 2009, 131:197-206.
46. Doran P., Martin G., Dowling P., Jockusch H., Ohlendieck K. Proteome analysis of the dystrophin-deficient mdx diaphragm reveals a drastic increase in the heat shock protein cvHSP. Proteomics 2006, 6:4610-4621.
47. Zanotti S., Negri T., Cappelletti C., et al. Decorin and biglycan expression is differentially altered in several muscular dystrophies. Brain 2005, 128:2546-2555.
48. Mauviel A. Transforming growth factor-beta: a key mediator of fibrosis. Methods Mol Med 2005, 117:69-80.
49. Nakao A., Imamura T., Souchelnytskyi S., et al. TGF-β receptor-mediated signalling through Smad2, Smad3 and Smad4. EMBO J 1997, 16:5353-5362.
50. Bernasconi P., Torchiana E., Confalonieri P., et al. Expression of transforming growth factor-beta 1 in dystrophic patient muscles correlates with fibrosis pathogenetic role of a fibrogenic cytokine. J Clin Invest 1995, 96:1137-1144.
51. Parsons C.J., Takashima M., Rippe R.A. Molecular mechanisms of hepatic fibrogenesis. J Gastroenterol Hepatol 2007, 22(SUPPL. 1):S79-S84.
52. Verrecchia F., Mauviel A. Transforming growth factor-beta and fibrosis. World J Gastroenterol 2007, 13:3056-3062.
53. Yu Q., Stamenkovic I. Cell surface-localized matrix metalloproteinase-9 proteolytically activates TGF-β and promotes tumor invasion and angiogenesis. Genes Dev 2000, 14:163-176.
54. Kim E.S., Kim M.S., Moon A. TGF-β-induced upregulation of MMP-2 and MMP-9 depends on p38 MAPK, but not ERK signaling in MCF10A human breast epithelial cells. Int J Oncol 2004, 25:1375-1382.
55. Lee E.O., Kang J.L., Chong Y.H. The amyloid-beta peptide suppresses transforming growth factor-beta1-induced matrix metalloproteinase-2 production via Smad7 expression in human monocytic THP-1 cells. J Biol Chem 2005, 280:7845-7853.
56. Shi X., Garry D.J. Muscle stem cells in development, regeneration, and disease. Genes Dev 2006, 20:1692-1708.
57. Charge S.B., Rudnicki M.A. Cellular and molecular regulation of muscle regeneration. Physiol Rev 2004, 84:209-238.
58. Hawke T.J., Garry D.J. Myogenic satellite cells: physiology to molecular biology. J Appl Physiol 2001, 91:534-551.
59. Weber M.S., Hohlfeld R., Zamvil S.S. Mechanism of action of Glatiramer acetate in treatment of multiple sclerosis. Neurotherapeutics 2007, 4:647-653.