Complement 3 deficiency and oral prednisolone improve strength and prolong survival of laminin α2-deficient mice
AM Connolly, RM Keeling, EM Streif, A Pestronk… - Journal of …, 2002 - Elsevier
Complement deposition and macrophages are common in biopsies of children with
muscular dystrophy. While the presumed roles of complement and macrophages have been
those of scavenger to remove and clear necrotic fibers, there is some evidence that they play
a primary role in the pathogenesis of these diseases. Here, we explore the role of
complement in the pathogenesis of the most severe animal model of congenital dystrophy,
the dy−/− mouse, which is laminin α2-deficient. We generated animals deficient in both C3 …
muscular dystrophy. While the presumed roles of complement and macrophages have been
those of scavenger to remove and clear necrotic fibers, there is some evidence that they play
a primary role in the pathogenesis of these diseases. Here, we explore the role of
complement in the pathogenesis of the most severe animal model of congenital dystrophy,
the dy−/− mouse, which is laminin α2-deficient. We generated animals deficient in both C3 …