Animal models of scleroderma: Lessons from transgenic and knockout mice

Current Opinion in Rheumatology

Volumn 21, Issue 6, 2009, Pages 630-635

  Derrett Smith, Emma C ^a   Denton, Christopher P ^a   Sonnylal, Sonali ^b  

^a ROYAL FREE AND UNIVERSITY COLLEGE MEDICAL SCHOOL   (United Kingdom)

^b UNIVERSITY OF TEXAS   (United States)

Author keywords

Animal models; Fibrosis; Scleroderma; Systemic sclerosis

Indexed keywords

CALCITONIN GENE RELATED PEPTIDE; COLLAGEN; IMATINIB; PLATELET DERIVED GROWTH FACTOR; SOMATOSTATIN; SUBSTANCE P; TAMOXIFEN; TRANSFORMING GROWTH FACTOR BETA; VANILLOID RECEPTOR 1;

DISEASE ASSOCIATION; FIBROBLAST; FIBROSIS; GENE EXPRESSION; HEMATOPOIETIC STEM CELL; HUMAN; KNOCKOUT MOUSE; LUNG FIBROSIS; LUNG INJURY; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; REVIEW; SCLERODERMA; SIGNAL TRANSDUCTION; SKIN FIBROSIS; SYSTEMIC SCLEROSIS; TRANSCRIPTION INITIATION; TRANSGENIC MOUSE; VASCULAR DISEASE;

ANIMALS; COLLAGEN; DISEASE MODELS, ANIMAL; FIBROBLASTS; FIBROSIS; HEMATOPOIETIC STEM CELLS; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; MODELS, BIOLOGICAL; NEUROPEPTIDES; PLATELET-DERIVED GROWTH FACTOR; SCLERODERMA, SYSTEMIC; SIGNAL TRANSDUCTION; TRANSFORMING GROWTH FACTOR BETA; VASCULAR DISEASES;

EID: 70349826650     PISSN: 10408711     EISSN: None     Source Type: Journal    
DOI: 10.1097/BOR.0b013e32833130c1     Document Type: Review

References (34)

1. Bou-Gharios G, Garrett LA, RossertJ, et al. A potent far-upstream enhancer in the mouse pro alpha 2(I) collagen gene regulates expression of reporter genes in transgenic mice. J Cell Biol 1996; 134:1333-1344.
2. Schuler M, Ali F, Metzger E, et al. Temporally controlled targeted somatic mutagenesis in skeletal muscles of the mouse. Genesis 2005; 41:165-170.
3. Zheng B, Zhang Z, Black CM, et al. Ligand-dependent genetic recombination in fibroblasts: a potentially powerful technique for investigating gene function in fibrosis. Am J Pathol 2002; 160:1609-1617.
4. Sonnylal S, Denton CP, Zheng B, et al. Postnatal induction of TGF-p signaling in fibroblasts of mice recapitulates clinical, histological and biochemical features of scleroderma. Arthritis Rheum 2007; 56:334-344.
5. Hoyles R, Khan K, Shiwen X, et al. Fibroblast-specific perturbation of trans forming growth factor beta signaling provides insight into potential pathogenic mechanisms of scleroderma-associated lung fibrosis: exaggerated response to alveolar epithelial injury in a novel mouse model. Arthritis Rheum 2008; 58:1175-1188.
6. Denton CP, Zheng B, Evans LA, et al. Fibroblast -specific expression of a kinase-deficient type II transforming growth factor p (TGFp) receptor leads to paradoxical activation of TGFp signaling pathways with fibrosis in transgenic mice. J Biol Chem 2003; 278:25109-25119.
7. Denton CP, Lindahl GE, Khan K, et al. Activation of key profibrotic mechanisms in transgenic fibroblasts expressing kinase-deficient type II transforming growth factor-p receptor. J Biol Chem 2005; 280:16053-16065.
8. Derrett-Smith EC, Khan K, Shi-wen X et al. Pulmonary vasculopathy in a TGF-p dependent mouse model of scleroderma. In: Proceedings of the Annual Scientific Meeting/Rheumatology Health Professionals (ACR/ARHP); 24-28 October 2008; San Francisco.
9. Denton CP, Khan K, Hoyles RK, et al. Inducible lineage-specific deletion of TbetaRII in fibroblasts defines a pivotal regulatory role during adult skin wound healing. J Invest Dermatol 2009; 129:194-204.
10. Zhao J, Sime PJ, Bringas P, et al. Epithelium-specific adenoviral transfer of a dominant-negative mutant TGF-b type II receptor stimulates embryonic lung branching morphogenesis in culture and potentiates EGF and PDGF-AA. Mech Dev 1998; 72:89-100.
11. Chen H, Zhuang F, Liu YH, et al. TGF-beta receptor II in epithelia versus mesenchyme plays distinct roles in the developing lung. Eur Respir J 2008, 32:285-295.
12. Boffa J-J, Tharaux P-L, Dussaule J-C, Chatziantoniou C. Regression of renal vascular fibrosis by endothelin receptor antagonism. Hypertension 2001; 37:490-496.
13. Roufosse C, Bou-Gharios G, Prodromidi E, et al. Bone marrow-derived cells do not contribute significantly to collagen I synthesis in a murine model of renal fibrosis. J Am Soc Nephrol 2006; 17:775-782.
14. Russo FP, Alison MR, Bigger BW, et al. The bone marrow functionally contributes to liver fibrosis. Gastroenterology 2006; 130:1807-1821.
15. RajkumarVS, Xu SW, Bostrom M, et al. Platelet-derived growth factor-beta receptor activation is essential for fibroblast and pericyte recruitment during cutaneous wound healing. Am J Pathol 2006; 169:2254-2265.
16. Sonnylal S, Bou-Gharios G, Abraham D, de Crombrugghe B. CTGF over-expressing mice: a model for human scleroderma. Matrix Biol 2006; 25:S32-S33.
17. Liu S, Kapoor M, LeaskA. Rac1 expression by fibroblasts is required for tissue repair in vivo. Am J Path 2009; 174:1847-1856.
18. Shi-Wen X, Chen Y, Denton CP, et al. Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/ Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol Cell Biol 2004; 15:2707-2719.
19. Kapoor M, Liu S, Shi-wen X, et al. GSK-3p in mouse fibroblasts controls wound healing and fibrosis through an endothelin-1-dependent mechanism. J Clin Invest 2008; 118:3279-3290.
20. Del Galdo F, Sotgia F, de Almeida CJ, et al. Decreased expression of caveolin 1 in patients with systemic sclerosis: crucial role in the pathogenesis of tissue fibrosis. Arthritis Rheum 2008; 58:2854-2865.
21. Maurer B, Busch N, Wagner EF, et al. Transgenic expression of the transcription factorfra-2 in mice induces progressive vascular injury and fibrosis of the skin resembling human SSc. In: Proceedings of the Annual Scientific Meeting/Rheumatology Health Professionals (ACR/ARHP); 24-28 October 2008; San Francisco.
22. Varga J, Pasche B. Transforming growth factor beta as a therapeutic target in systemic sclerosis. Nat Rev Rheumatol 2009; 5:200-206; doi: 10.1038/ nrrheum.2009.26.
23. Katebi M, Fernandez P, Chan ES, Cronstein BN. Adenosine A2A receptor blockade or deletion diminishes fibrocyte accumulation in the skin in a murine model of scleroderma, bleomycin-induced fibrosis. Inflammation 2008; 31: 299-303.
24. Boban I, Barisic-Dujmovic T, Clark SH. Parabiosis and transplantation models show no evidence of circulating dermal fibroblast progenitors in bleomycin-induced skin fibrosis. J Cell Physiol 2008; 214:230-237.
25. Akhmetshina A, Venalis P, Dees C, et al. Treatment with imatinib prevents fibrosis in different preclinical models of systemic sclerosis and induces regression of established fibrosis. Arthritis Rheum 2009; 60: 219-224.
26. Olsen LE, Soriano P. Increased PDGFRalpha activation disrupts connective tissue development and drives systemic fibrosis. Dev Cell 2009; 16:303-313.
27. Razavi R, Chan Y, Afifiyan FN, et al. TRPV1 + sensory neurons control beta cell stress and islet inflammation in autoimmune diabetes. Cell 2006; 127:1123-1135.
28. Barnes PJ. Asthma as an axon reflex. Lancet 1986; 1:242-245.
29. Gutwald J, Goebeler M, Sorg CJ. Neuropeptides enhance irritant and allergic contact dermatitis. J Invest Dermatol 1991; 96:695-698.
30. Keeble J, Russell F, Curtis B, et al. Involvement of transient receptor potential vanilloid 1 in the vascular and hyperalgesic components of joint inflammation. Arthritis Rheum 2005; 52:3248-3256.
31. Szabo A, Czirjak L, Sandor Z, et al. Investigation of sensory neurogenic components in a bleomycin-induced scleroderma model using transient receptor potential vanilloid 1 receptor- and calcitonin gene-related peptide-knockout mice. Arthritis Rheum 2008; 58:292-301.
32. Asano Y, Markiewicz M, Kubo M, et al. Transcription factor Fli1 regulates collagen fibrillogenesis in mouse skin. Mol Cell Biol 2009; 29:425-434.
33. Czuwara-Ladykowska J, Shirasaki F, Jackers P, et al. Fli-1 inhibits collagen type I production in dermal fibroblasts via an Sp1-dependent pathway. J Biol Chem 2001; 276:20839-20848.
34. Muro AF, Moretti FA, Moore BB, et al. An essential role for fibronectin extra type III domain A in pulmonary fibrosis. Am J Resp Crit Care Med 2008; 177: 638-645.