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]

TβR1CA; 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. TβR1CA; 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.

Gilbane et al. Arthritis Research & Therapy 2013 15:215   doi:10.1186/ar4230