Thursday, November 7, 2013
This week in techniques
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Fibrillin 1 (Fbn1)-mutant mouse models
of systemic scleroderma
Mice harboring mutations in
the integrin-binding domain of Fbn1 could help model systemic scleroderma.
The mice exhibited multiple disease features seen in patients with systemic
scleroderma or patient fibroblasts, including high levels of collagen,
activated integrin b3
(GPIIIa; CD61) in the skin and high
levels of antinuclear antibodies in circulation. Compared with control IgG,
an integrin b1
decreased CD61 activity and collagen expression in patient fibroblasts, skin
fibrosis and levels of circulating antinuclear antibodies in the mice. Future
studies could include using the models to compare the efficacy and safety of
CD29-activating and CD61-inhibiting antibodies.
Mitsubishi Tanabe Pharma Corp.'s
Venoglobulin IH (GB-0998), a liquid human
IgG preparation derived from donated plasma, is in Phase III testing to treat
arGentis Pharmaceuticals LLC's
ARG201, a solubilized type
I native bovine collagen, is in Phase II testing to treat systemic
Published online Nov. 7, 2013
Patent and licensing status
Gerber, E.E. et al.
Nature; published online Oct. 9, 2013;
Contact: Harry C. Dietz, The Johns Hopkins University School
of Medicine, Baltimore, Md.
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