This Project Grant award from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), under the Arthritis, Musculoskeletal and Skin Diseases Research program (CFDA 93.846), supports research into the molecular mechanisms underlying fibrosis and disease progression in recessive dystrophic epidermolysis bullosa (RDEB). The $343,200 award, with a period of performance from June 1, 2024 to May 31, 2029, will enable researchers at Thomas Jefferson University to investigate how the loss of type VII collagen disrupts protein secretion pathways in RDEB, leading to increased TGF-beta signaling and fibrosis. The project will also assess the potential of anti-viral drugs to normalize secretion defects and reduce fibrosis in RDEB, with the goal of developing new therapeutic approaches. This research aims to provide critical insights into the molecular basis of fibrosis in RDEB and other fibrotic diseases, and to identify novel treatment strategies that can be translated into clinical trials.
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