The Agnetti Lab
Center for Research on Cardiac Intermediate Filaments
We work to elucidate the pathophysiological role of the intermediate filaments cytoskeleton in cardiovascular disease, with an emphasis on desmin modifications and misfolding.
Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes
(iPSC-CM) in action - collaboration with the Tampakakis lab at JHU
New roles for desmin in the maintenance of muscle homeostasis
Desmin is the primary intermediate filament (IF) of cardiac, skeletal, and smooth muscle. By linking the contractile myofibrils to the sarcolemma and cellular organelles, desmin IF contributes to muscle structural and cellular integrity, force transmission, and mitochondrial homeostasis. Mutations in desmin cause myofibril misalignment, mitochondrial dysfunction, and impaired mechanical integrity leading to cardiac and skeletal myopathies in humans, often characterized by the accumulation of protein aggregates. Recent evidence indicates that desmin filaments also regulate proteostasis and cell size. In skeletal muscle, changes in desmin filament dynamics can facilitate catabolic events as an adaptive response to a changing environment. In addition, post-translational modifications of desmin and its misfolding in the heart have emerged as key determinants of homeostasis and disease. In this review, we provide an overview of the structural and cellular roles of desmin and propose new models for its novel functions in preserving the homeostasis of striated muscles.
Art with a Heart
Special thank you to Laura for helping us with our lab's logo and doing fantastic art all around!
Overnight Time-Lapse of NRVMs expressing GFP after being transduced with AAV6.2