Speaker: Dr. Aaron Beedle (SUNY Binghamton University)
Where: L-GE 110 | When: 1:15 pm “Mechanistic and Therapeutic Advances in Dystroglycan Glycosylation-Deficient Muscular Dystrophy” Abstract: The focus of the Beedle lab is to develop and analyze cellular and animal models of dystroglycan glycosylation-deficient muscular dystrophy, the “dystroglycanopathies”, to gain mechanistic insight into the disease process and to develop therapeutic strategies. Current projects include muscle regenerative capacity in muscular dystrophy and preclinical therapeutic trials targeting cellular signaling pathways. The mammalian target of rapamycin (mTOR) complex 1 is a kinase complex downstream of phosphoinositide 3 kinase (PI3K) and protein kinase B (Akt) signaling, with important roles in cellular growth, metabolism, and autophagy. We find abnormal hyperactivation of this pathway in skeletal muscle from dystroglycanopathy models. Pharmacological studies targeting the mTORC1 complex indicate that mTORC1 hyperactivation promotes disease progression and/or severity using behavioural, biochemical, and histological outcomes. A preclinical trial is now underway to assess the efficacy and toxicity of mTORC1 inhibitors in dystroglycanopathy muscular dystrophy. Photos By: Michael Chung (left); Arun Dayanandan (right) Comments are closed.
|
BGSA NewsLatest News Archives
September 2018
Categories |