2013 Student Research Conference:
26th Annual Student Research Conference

Examination of Revertant Muscle Fiber Number Variance in Dystrophin-Null Mice of Various Mutations and Genetic Backgrounds
Thomas P. McDonald
Dr. Dongsheng Duan (University of Missouri) and Dr. George L. Shinn, Faculty Mentors

Duchenne muscular dystrophy (DMD) is a severe muscle wasting disorder caused by mutations in the DMD gene, abolishing dystrophin protein expression. Surprisingly, rare-occurring dystrophin positive myofibers, known as revertant fibers, have been found in patients and animal models of DMD. A better understanding of the molecular and cellular factors that regulate the formation of revertant fibers may reveal novel therapeutic avenues. Here we examine the effect of genetic background, mutation site, and muscle type on the occurrence of revertant fibers in various dystrophin-null mice. DMD model mdx mice (BL10 genetic background) were bred to the FVB and BL6 backgrounds through six generations of backcrossing. BL6 background mdx3cv and mdx4cv mice were also included in the study. Revertant fiber frequency in the tibialis anterior, soleus, diaphragm and heart of 3-month-old male mice were blindly quantified using two antibodies that recognize either the N-terminal or the C-terminal end of the dystrophin protein.

Keywords: Duchenne muscular dystrophy, dystrophin, revertant fiber, mdx , mdx3cv, mdx4cv, genetic background


Presentation Type: Oral Paper

Session: 109-3
Location: MG 2001
Time: 8:30

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