ARTICLE | Tools & Techniques
Scleroderma models: skin in the game
November 14, 2013 8:00 AM UTC
Systemic scleroderma involves fibrosis of the skin and internal organs, but its etiology is poorly understood and it has no known genetic causes-factors that have hampered the development of models and, in turn, therapies.
Now, a North American team has shown that mice harboring a mutant form of the glycoprotein fibrillin 1 (Fbn1) recapitulated skin fibrosis and other symptoms seen in patients with systemic scleroderma.1 Although the team showed the therapeutic effect of targeting different proteins whose expression was altered by the mutant glycoprotein, future studies will have to zero in on the best target for treating scleroderma in patients...