Duchenne muscular dystrophy fibroblast nodules: A cell-based assay for screening anti-fibrotic agents

Cell and Tissue Research

Volumn 352, Issue 3, 2013, Pages 659-670

  Zanotti, Simona ^a   Gibertini, Sara ^a   Savadori, Paolo ^a   Mantegazza, Renato ^a   Mora, Marina ^a  

^a DEPARTMENT OF NEUROSURGERY   (Italy)

Author keywords

Cell culture (Human); Decorin; Fibroblast; Muscle fibrosis; Spironolactone; Suramin

Indexed keywords

COLLAGEN TYPE 1; DECORIN; FIBRONECTIN; SPIRONOLACTONE; SURAMIN;

ARTICLE; CHILD; CLINICAL ARTICLE; COLLAGEN SYNTHESIS; CONCENTRATION RESPONSE; CONTROLLED STUDY; DRUG EFFECT; DRUG SCREENING; DUCHENNE MUSCULAR DYSTROPHY; EXTRACELLULAR MATRIX; FIBROBLAST; FIBROBLAST CULTURE; FIBROGENESIS; HUMAN; HUMAN CELL; IN VITRO STUDY; MONOLAYER CULTURE; PRESCHOOL CHILD; PRIORITY JOURNAL; PROTEIN EXPRESSION;

BIOLOGICAL ASSAY; BLOTTING, WESTERN; COLLAGEN; DECORIN; DRUG EVALUATION, PRECLINICAL; ENZYME-LINKED IMMUNOSORBENT ASSAY; FIBROBLASTS; FIBRONECTINS; FIBROSIS; GENE EXPRESSION REGULATION; HUMANS; MUSCULAR DYSTROPHY, DUCHENNE; RNA, MESSENGER; SPIRONOLACTONE; SURAMIN;

EID: 84878592126     PISSN: 0302766X     EISSN: 14320878     Source Type: Journal    
DOI: 10.1007/s00441-013-1601-2     Document Type: Article

References (50)

1. Banker BQ, Engel AG (2004) Basic reactions of muscle. In: Engel AG, Franzini-Armstrong C (eds) Myology. McGraw-Hill, New York, pp 691-748
2. Baudino TA, Carver W, Giles W, Borg TK (2006) Cardiac fibroblasts: friend or foe? Am J Physiol Heart Circ Physiol 291:1015-1026
3. Bernasconi P, Torchiana E, Confalonieri P, Brugnoni R, Barresi R, Mora M, Cornelio F, Morandi L, Mantegazza R (1995) Expression of transforming growth factor-β1 in dystrophic patient muscles correlates with fibrosis: pathogenetic role of a fibrogenic cytokine. J Clin Invest 96:1137-1144
4. Chamberlain J, Shah M, Ferguson MW (1995) The effect of suramin on healing adult rodent dermal wounds. J Anat 186:87-96
5. Chan YS, Li Y, Foster W, Horaguchi T, Somogyi G, Fu FH, Huard J (2003) Anti-fibrotic effects of suramin in injured skeletal muscle after laceration. J Appl Physiol 95:771-780
6. Chan YS, Li Y, Foster W, Fu FH, Huard J (2005) The use of suramin, an anti-fibrotic agent, to improve muscle recovery after strain injury Am J Sports Med 33:43-51
7. Chang HY, Chi JT, Dudoit S, Bondre C, M van de Rijn, Botstein D, Brown PO (2002) Diversity, topographic differentiation, and positional memory in human fibroblasts Proc Natl Acad Sci USA 99:12877-12882
8. Coffey RJ Jr, Leof EB, Shipley GD, Moses HL (1987) Suramin inhibition of growth factor receptor binding and mitogenicity in AKR-2B cells. J Cell Physiol 132:143-148
9. Desmoulière A, Chaponnier C, Gabbiani G (2005) Tissue repair, contraction, and the myofibroblast. Wound Repair Regen 13:7-12
10. Detlefsen S, Sipos B, Feyerabend B, Klöppel G (2005) Pancreatic fibrosis associated with age and ductal papillary hyperplasia. Virchows Arch 447:800-805
11. Fleischmajer R, Perlish JS, Krieg T, Timpl R (1981) Variability in collagen and fibronectin synthesis by scleroderma fibroblasts in primary culture. J Invest Dermatol 76:400-403
12. Fukushima K, Badlani N, Usas A, Riano F, Fu F, Huard J (2001) The use of an antifibrosis agent to improve muscle recovery after laceration. Am J Sports Med 29:394-402
13. Gimbrone MA Jr, Cotran RS (1975) Human vascular smooth muscle in culture. Growth and ultrastructure. Lab Invest 33:16-27
14. Glass WF 2nd, Teng PR, Haney LB (1996) Extracellular matrix distribution and hillock formation in human mesangial cells in culture without serum. J Am Soc Nephrol 7:2230-2243
15. Guney I, Selcuk NY, Altintepe L, Atalay H, Başarali MK, Büyükbaş S (2009) Anti-fibrotic effects of aldosterone receptor blocker (spironolactone) in patients with chronic kidney disease. Ren Fail 31:779-784
16. Guyot C, Lepreux S, Combe C, Doudnikoff E, Bioulac-Sage P, Balabaud C, Desmoulière A (2006) Hepatic fibrosis and cirrhosis: the (myo)fibroblastic cell subpopulations involved. Int J Biochem Cell Biol 38:135-151
17. Hinz B (2007) Formation and function of the myofibroblast during tissue repair. J Invest Dermatol 127:526-537
18. Hu Q, Noor M, Wong YF, Hylands PJ, Simmonds MS, Xu Q, Jiang D, Hendry BM, Xu Q (2009) In vitro anti-fibrotic activities of herbal compounds and herbs. Nephrol Dial Transplant 24:3033-3041
19. Huard J, Li Y, Fu F (2002) Muscle injury and repair: current trends in research. J Bone Joint Surg Am 84:822-832
20. Jordana M, Schulman J, McSharry C, Irving LB, Newhouse MT, Jordana G, Gauldie J (1988) Heterogeneous proliferative characteristics of human adult lung fibroblast lines and clonally derived fibroblasts from control and fibrotic tissue. Am Rev Respir Dis 137:579-584
21. King TE Jr, Pardo A, Selman M (2011) Idiopathic pulmonary fibrosis. Lancet 378:1949-1961
22. Lane A, Johnson DW, Pat B, Winterford C, Endre Z, Wei M, Gobe GC (2002) Interacting roles of myofibroblasts, apoptosis and fibrogenic growth factors in the pathogenesis of renal tubulo-interstitial fibrosis. Growth Factors 20:109-119
23. Li Y, Cummins J, Huard J (2001) Muscle injury and repair. Curr Opin Orthop 12:409-415
24. Li Y, Li J, Zhu J, Sun B, Branca M, Tang Y, Foster W, Xiao X, Huard J (2007) Decorin gene transfer promotes muscle cell differentiation and muscle regeneration. Mol Ther 15:1616-1622
25. Liu N, Tolbert E, Ponnusamy M, Yan H, Zhuang S (2011) Delayed administration of suramin attenuates the progression of renal fibrosis in obstructive nephropathy. J Pharmacol Exp Ther 338:758-766
26. Moller-Pederson T, Cavanag HD, Petroll WM, Jester J (1998) Neutralizing antibody to TGF-β moderates stromal fibrosis but not regression of photoablative effect following PRK. Curr Eye Res 17:736-747
27. Nozaki M, Li Y, Zhu J, Ambrosio F, Uehara K, Fu FH, Huard J (2008) Improved muscle healing after contusion injury by the inhibitory effect of suramin on myostatin, a negative regulator of muscle growth. Am J Sports Med 36:2354-2362
28. Pannu J, Gardner H, Shearstone JR, Smith E, Trojanowska M (2006) Increased levels of transforming growth factor beta receptor type I and up-regulation of matrix gene program: a model of scleroderma. Arthritis Rheum 54:3011-3021
29. Poobalarahi F, Baicu CF, Bradshaw AD (2006) Cardiac myofibroblasts differentiated in 3D culture exhibit distinct changes in collagen I production, processing, and matrix deposition. Am J Physiol Heart Circ Physiol 291:H2924-H2932
30. Powell DW, Mifflin RC, Valentich JD, Crowe SE, Saada JI, West AB (1999) Myofibroblasts. I. Paracrine cells important in health and disease. Am J Physiol 277:C1-C9
31. Rafael-Fortney JA, Chimanji NS, Schill KE, Martin CD, Murray JD, Ganguly R, Stangland JE, Tran T, Xu Y, Canan BD, Mays TA, Delfín DA, Janssen PM, Raman SV (2011) Early treatment with lisinopril and spironolactone preserves cardiac and skeletal muscle in Duchenne muscular dystrophy mice. Circulation 124:582-588
32. Raghu G, Chen YY, Rusch V, Rabinovitch PS (1988) Differential proliferation of fibroblasts cultured from normal and fibrotic human lungs. Am Rev Respir Dis 138:703-708
33. Ramos C, Montano M, Garcia-Alvarez J, Ruiz V, Uhal BD, Selman M, Pardo A (2001) Fibroblasts from idiopathic pulmonary fibrosis and normal lungs differ in growth rate, apoptosis, and tissue inhibitor of metalloproteinases expression. Am J Respir Cell Mol Biol 24:591-598
34. Sarrazy V, Billet F, Micallef L, Coulomb B, Desmoulière A (2011) Mechanisms of pathological scarring: role of myofibroblasts and current developments. Wound Repair Regen 19 (Suppl 1):10-15
35. Schindler M, Ahmed I, Kamal J, Nur-E-Kamal A, Grafe TH, Young Chung H, Meiners S (2005) A synthetic nanofibrillar matrix promotes in vivo-like organization and morphogenesis for cells in culture. Biomaterials 26:5624-5631
36. Stein CA (1993) Suramin: a novel antineoplastic agent with multiple potential mechanisms of action. Cancer Res 53:2239-2248
37. Sterzel RB, Lovett DH, Foellmer HG, Perfetto M, Biemesderfer D, Kashgarian M (1986) Mesangial cell hillocks. Nodular foci of exaggerated growth of cells and matrix in prolonged culture. Am J Pathol 125:130-140
38. Taniguti AP, Pertille A, Matsumura CY, Santo Neto H, Marques MJ (2011) Prevention of muscle fibrosis and myonecrosis in mdx mice by suramin, a TGF-beta1 blocker. Muscle Nerve 43:82-87
39. Tian J, Shidyak A, Periyasamy SM, Haller S, Taleb M, El-Okdi N, Elkareh J, Gupta S, Gohara S, Fedorova OV, Cooper CJ, Xie Z, Malhotra D, Bagrov AY, Shapiro JI (2009) Spironolactone attenuates experimental uremic cardiomyopathy by antagonizing marinobufagenin. Hypertension 54:1313-1320
40. Tiggelman AM, Linthorst C, Boers W, Brand HS, Chamuleau RA (1997) Transforming growth factor-beta-induced collagen synthesis by human liver myofibroblasts is inhibited by alpha2-macroglobulin. J Hepatol 26:1220-1228
41. Tomasini-Johansson BR, Johnson IA, Hoffmann FM, Mosher DF (2012) Quantitative microtiter fibronectin fibrillogenesis assay: use in high throughput screening for identification of inhibitor compounds. Matrix Biol 31:360-367
42. Trachtman H, Weiser AC, Valderrama E, Morgado M, Palmer LS (2004) Prevention of renal fibrosis by spironolactone in mice with complete unilateral ureteral obstruction. J Urol 172:1590-1594
43. Verrecchia F, Mauviel A (2002) Transforming growth factor-beta signaling through the Smad pathway: role in extracellular matrix gene expression and regulation. J Invest Dermatol 118:211-215
44. Wynes MW, Edelman BL, Kostyk AG, Edwards MG, Coldren C, Groshong SD, Cosgrove GP, Redente EF, Bamberg A, Brown KK, Reisdorph N, Keith RC, Frankel SK, Riches DW (2011) Increased cell surface Fas expression is necessary and sufficient to sensitize lung fibroblasts to Fas ligation-induced apoptosis: implications for fibroblast accumulation in idiopathic pulmonary fibrosis. J Immunol 187:527-537
45. Xu Q, Norman JT, Shrivastav S, Lucio-Cazana J, Kopp JB (2007) In vitro models of TGF-beta-induced fibrosis suitable for high-throughput screening of anti-fibrotic agents. Am J Physiol Renal Physiol 293:F631-F640
46. Yokozeki M, Moriyama K, Shimokawa H, Kuroda T (1997) Transforming growth factor-beta 1 modulates myofibroblastic phenotype of rat palatal fibroblasts in vitro. Exp Cell Res 231:328-336
47. Zanotti S, Saredi S, Ruggieri A, Fabbri M, Blasevich F, Romaggi S, Morandi L, Mora M (2007) Altered extracellular matrix transcript expression and protein modulation in primary Duchenne muscular dystrophy myotubes. Matrix Biol 26:615-624
48. Zanotti S, Gibertini S, Mora M (2010) Altered production of extra-cellular matrix components by muscle-derived Duchenne muscular dystrophy fibroblasts before and after TGF-beta1 treatment. Cell Tissue Res 339:397-410
49. Zanotti S, Gibertini S, Bragato C, Mantegazza R, Morandi L, Mora M (2011a) Fibroblasts from the muscles of Duchenne muscular dystrophy patients are resistant to cell detachment apoptosis. Exp Cell Res 317:2536-2547
50. Zanotti S, Gibertini S, Di Blasi C, Cappelletti C, Bernasconi P, Mantegazza R, Morandi L, Mora M (2011b) Osteopontin is highly expressed in severely dystrophic muscle and seems to play a role in muscle regeneration and fibrosis. Histopathology 59:1215-1228