ADAMTSL2 regulates Wnt signaling in myogenesis

ADAMTSL2 mutations cause geleophysic dysplasia, which is characterized by short stature, pseudomuscular build, joint stiffness and tight skin. To elucidate the role of ADAMTSL2 in skeletal muscle myogenesis, we used C2C12 myoblasts, which differentiate and form myotubes when serum is reduced as a model system for myogenesis. Adamtsl2 was depleted by stably expressing shRNA targeting Adamtsl2 mRNA. Upon shRNA-mediated depletion of Adamtsl2, C2C12 myoblasts did not differentiate and did not form myosin heavy chain-positive myotubes in cell culture. Comparison of control and Adamtsl2 depleted C2C12 cells 3 days after onset of differentiation. Adamtsl2 was depleted by stable shRNA overexpression in C2C12 myoblasts. Differentiation was induced by serum-reduction. 3 replicates were analyzed each for control and Adamtsl2 knockdown.