Biomathematics Seminar: Dynamics of the Muscular-Dystrophy Protein in Muscle Fibre Cells
17 January 2012 14:00 in Department of Mathematical Sciences
Mutations in the Dystrophin (Dys) gene are the cause of muscular dystrophy,
yet relatively little is known about its behaviour and function. Muscle cells in tissue without the Dys protein are known to have tears in the plasma membranes, and to die, but how an absence of Dys causes this is not known. The absence of Dys causes Duchenne muscular dystrophy. Only cells in functioning muscle tissue express Dys and so it can only be studied in live animals (not in cell culture). We have studied fluorescently-tagged Dys in muscle fibre cells in living zebrafish embryos. We studied its dynamics by combining confocal microscopy with quantitative modelling. Dys appears to move via simple diffusion in the cells with a diffusion constant of order 2 micron2/s. This is surprisingly large; the protein Dystrophin is huge (3919 amino acids, 10 times larger than the average protein). Dys also binds at the tips of the cells where its dynamics is reaction-controlled, with a bound lifetime of around 4 minutes. We believe this is the biggest protein whose dynamics have been studied quantitatively in living cells. I will discuss the consequences of these findings for the function of Dys and for future treatments for muscular dystrophy. Work done by Fernanda Bajanca and Simon Hughes (KCL), and Vinicio Gonzalez-Perez and RPS (Surrey).
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