A Mathematical Model of Skeletal Muscle Disease and Immune Response in themdxMouse
Author(s) -
Abdul Salam Jarrah,
Filippo Castiglione,
Nicholas P. Evans,
Robert W. Grange,
Reinhard Laubenbacher
Publication year - 2014
Publication title -
biomed research international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 126
eISSN - 2314-6141
pISSN - 2314-6133
DOI - 10.1155/2014/871810
Subject(s) - mdx mouse , duchenne muscular dystrophy , immune system , muscular dystrophy , disease , genetic model , biology , skeletal muscle , clearance , immunology , regeneration (biology) , animal model , medicine , pathology , dystrophin , microbiology and biotechnology , anatomy , genetics , endocrinology , gene , urology
Duchenne muscular dystrophy (DMD) is a genetic disease that results in the death of affected boys by early adulthood. The genetic defect responsible for DMD has been known for over 25 years, yet at present there is neither cure nor effective treatment for DMD. During early disease onset, the mdx mouse has been validated as an animal model for DMD and use of this model has led to valuable but incomplete insights into the disease process. For example, immune cells are thought to be responsible for a significant portion of muscle cell death in the mdx mouse; however, the role and time course of the immune response in the dystrophic process have not been well described. In this paper we constructed a simple mathematical model to investigate the role of the immune response in muscle degeneration and subsequent regeneration in the mdx mouse model of Duchenne muscular dystrophy. Our model suggests that the immune response contributes substantially to the muscle degeneration and regeneration processes. Furthermore, the analysis of the model predicts that the immune system response oscillates throughout the life of the mice, and the damaged fibers are never completely cleared.
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