In a December 2012 podcast from Nationwide Children's Hospital, cell biologist Federica Montanaro discusses her team's recent progress in understanding how various proteins interact with dystrophin and how these interactions differ in the heart versus the skeletal muscles.
The approximately 15-minute podcast is based on a paper by Montanaro and colleagues, published online Aug. 24, 2012, in PLOS One. (See Proteomic Analysis Reveals New Cardiac-Specific Dystrophin-Associated Proteins.) A transcript will be provided along with the podcast on the Nationwide site in the section called This Month in Muscular Dystrophy.
Montanaro is a principal investigator in the Center for Gene Therapy at Nationwide Children's Hospital in Columbus, Ohio, and is an assistant professor in the Department of Pediatrics at the Research Institute at Nationwide Children's and at Ohio State University.
She's interviewed by Kevin Flanigan, a specialist in neuromuscular disorders and a principal investigator in the Center for Gene Therapy at Nationwide. Flanigan also co-directs the MDA neuromuscular disease clinic at that institution.
Montanaro describes how powerful new techniques used in molecular biology have allowed researchers to tease out exactly which proteins stick to ("bind") other proteins in a cell and how this technology has allowed greater understanding of dystrophin's usual role in the heart and skeletal muscles and what happens in dystrophin deficiency.
Montanaro says she hopes these new techniques will "fill in the gap in knowledge" of what happens between the loss of dystrophin and the death of a cell. Researchers, she says, know where the process "starts and where it ends" but that what happens in between has been "sort of a black box."
Better understanding of the "black box" could lead to trying existing or developing new drugs to compensate for dystrophin deficiency.
Specifically, Montanaro and colleagues have found that: