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Our research focuses on studying disorders of the diaphragm and other breathing muscles. The respiratory muscles pump air into and out of the lungs, and when these muscles are defective this can lead to respiratory failure and even death. Some disorders of the respiratory muscles are genetic in origin. This is the case for muscular dystrophies such as Duchenne Muscular Dystrophy and many other inherited diseases of skeletal muscle. In other patients, weakness of the respiratory muscles is not genetically determined but instead acquired due to another illness. This is especially frequent in critically ill individuals who become dependent upon mechanical ventilation in the intensive care unit, due to a condition called Ventilator-Induced Diaphragmatic Dysfunction. We are investigating the cellular and molecular mechanisms which contribute to these diseases, with an emphasis on translating these changes to the overall the development of respiratory muscle weakness and failure in patients.. THERA*EUTIC MODULATION OF INNATE IMMUNITY IN GENETIC MUSCLE DISEASE INVOLVING THE DIAPHRAGM: We have shown that modulation of innate immune mechanisms (e.g., chemokine receptors or Toll-like receptors) leads to dramatic improvements in contractile function as well as reduced muscle fibrosis in the standard murine model of Duchenne Muscular Dystrophy (DMD), the mdx mouse. The mdx mouse model is a genetic homologue of human DMD and our studies have largely focused on the diaphragm because it is the most severely affected muscle. Macrophages constitute by far the predominant inflammatory cell type within DMD and mdx muscles, where they exhibit different phenotypic profiles typified by “polarization” towards inflammatory (M1) or additionally modulate or be modulated by macrophage function. Our basic premise is that effective muscle repair depends upon maintaining that immune modulation by drug therapy needs to be specifically tailored to disease stage, guided by novel immune-based biomarkers.