Therapeutic Role of Dietary Lipids in LGMD2B Muscular Dystrophy
Dysferlin is hypothesized to mediate the rapid repair of the sarcolemma following muscle damage. Membrane biophysics teaches us that resistance to damage will be a function of the lipid composition of the sarcolemma. One therapeutic approach is to treat patients with compounds that fortify the natural tendency of membranes to reseal after damage, and minimize the inflammation that impedes regeneration and amplifies tissue destruction.
Since a significant part of our membrane lipids are derived from diet and dysferlin is hypothesized to interact with lipids, it is reasonable to try to bypass the requirement for dysferlin by altering the lipid composition of the sarcolemma to one that further promotes membrane resealing. Also, associated with an absence of dysferlin is impairment in purinergic signaling between muscle cells. We are investigating the relationships between membrane damage, purinergic signaling, and membrane composition to further understand the role of dysferlin in mediating membrane repair.