MDA awarded a research grant totaling $425,952 over a period of three years to Robert Korneluk, director of the Apoptosis Research Centre at the Children’s Hospital of Eastern Ontario Research Institute, and distinguished professor at the University of Ottawa in Ontario, Canada.

The funds will help support Korneluk’s study of the Nuclear Factor kappaB (NFkB) signaling pathway in muscle diseases such as Duchenne muscular dystrophy.

The NFkB signaling pathway is critical for normal skeletal muscle function, and is important for promoting recovery in response to muscle injury. There also is strong evidence that NFkB signaling is involved in the disease process of many muscle diseases.

Korneluk and colleagues recently found that two proteins called cIAP1 and cIAP2, initially identified by the group, are required for various aspects of NFkB signaling in skeletal muscle. The investigators also found that the loss of cIAP1 protein activity in muscle, in cultured cells and in mice, leads to changes in the NFkB signaling pathway, with subsequent improvement of muscle function and recovery.

Korneluk’s team plans to define the roles and mechanism of action of cIAP1 and cIAP2 in NFkB signaling in skeletal muscle, and evaluate their contribution to the DMD disease process. Additionally, the investigators will evaluate the therapeutic potential in muscular dystrophy of several drugs that specifically and strongly target cIAP1 and cIAP2 for destruction.

Funding for this MDA grant began February 1, 2012.