Research Assistant Professor
Boston University School of Medicine, Department of Neurology
My particular research interest is the roles of molecular chaperones and the ubiquitin-proteasome system in skeletal muscle diseases. During my postdoctoral training, I identified a new pathway of myogenic cell death in myofibrillar myopathy by generating and analyzing a knockout mouse for the co-chaperone bag3 (Bcl-2-associated anthanogene 3). Our finding led to the discovery of bag3 gene mutations in human myofibrillar myopathy and dilated cardiomyopathy.
I began working on FSHD in 2008 and have found that disrupted protein homeostasis is induced upon DUX4 expression. My research goals are to identify the mechanisms of DUX4-induced disrupted protein homeostasis and to develop therapeutic strategies to inhibit DUX4-induced muscle atrophy/dystrophy in FSHD.