Scleroderma pathogenesis: a pivotal role for fibroblasts as effector cells

Table 1 Murine models of scleroderma fi broblast dysfunction

SSc murine model	Model	Skin	Lung	Kidney	Heart	Gut	Vessels
Transgenic	TβRIIΔK	✓ [42]	✓ [42]			✓ [52]	✓ [43]
	TβR1^CA; Cre-ER	✓ [53]	✓ [53]	✓ [53]			✓ [53]
	COL1α2- CTGF	✓ [54]	✓ [54]	✓ [54]			✓ [54]
	Fra-2	✓ [47]	✓ [47]		✓ [55]
	Caveolin-1	✓ [56, 57]	✓ [56, 57]				✓ [56, 57]
	Relaxin	✓ [58]	✓ [58]	✓ [58]	✓ [58]
	Wnt10b	✓ [44]
Spontaneous	Tsk-1	✓ [59]	✓ [59]
	Tsk-2	✓ [60]
Chemical injury	Bleomycin	✓ [61]	✓ [19]
	HOCI	✓ [62]	✓ [62]

Comparison of mouse models of SSc that exhibit fibroblast-driven pathophysiology. TβRIIΔK, kinase deficient type-II transforming growth factor-β receptor mouse. TβR1^CA; Cre-ER, constitutively active transforming growth factor-β receptor 1 mouse. COL1α2-CTGF, collagen type-1, alpha-2, connective tissue growth factor mouse. Fra-2, fos-related antigen 2 transgenic mouse. Caveolin-1, caveolin-1-deficient mouse. Relaxin, relaxin knockout mouse. Wnt10b, Wnt10b overexpressing transgenic mouse. Tsk-1, tight-skin mouse 1. Tsk-2, tight skin mouse 2. HOCl, hypochlorous acid mouse model.

ISSN: 1478-6362