EM.P.3.05 Expression of fibulins 1–5 during myogenesis in vitro and in skeletal muscle regenerating in vivo, and in dystrophic mdx muscles

Abstract

Many myopathies are due to gene defects that result in absent or altered extracellular matrix (ECM) and numerous ECM molecules are in intimate contact with myofibres and with the quiescent muscle precursors (satellite cells). Complex interactions between ECM components and different cells play a crucial role in muscle function and all aspects of new muscle formation after damage. Fibulins (FBLN) are members of an adhesive glycoprotein family that are associated with fibrosis in some conditions. The role of FBLN 1–5 was investigated using immunostaining and qPCR in cultured myogenic C2C12 cells, in tissue sections of regenerating whole muscle grafts, in dystrophic (mdx) quadriceps and diaphragm, and in normal adult (2 month) and geriatric (23 month) mouse muscle.