Telomerase and Duchenne muscular dystrophy – RevGenetics

Telomerase and Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is a common inherited disorder that causes progressive muscle degeneration leading to premature death. One of the problems studying this disease involves a lack of a suitable organism to study the disease. One animal model commonly used is a mouse model (called mdx mutant mice) that also has the DMD mutated gene version but unfortunately its does not accurately represent the human version of this disease, that is, until now. A group from Stanford University created an improved version of the mouse model that more closely resembles the human disorder by removing the telomerase activity (Cell, Volume 143, December 2010, pages 1040-1042). This new animal model should help increase our understanding of the disease and equally important provide a new venue for testing treatments for this disease.

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