Monday, June 22, 2015

New fly model related to myotonic dystrophy type 1 (DM1)

Bargiela A, Cerro-Herreros E, Fernandez-Costa JM, Vilchez JJ, Llamusi B, Artero R. Increased autophagy and apoptosis contribute to muscle atrophy in a myotonic dystrophy type 1 Drosophila model. Dis Model Mech. 2015 Jul 1;8(7):679-90. PMID: 26092529.

From the abstract: "Muscle mass wasting is one of the most debilitating symptoms of myotonic dystrophy type 1 (DM1) disease, ultimately leading to immobility, respiratory defects, dysarthria, dysphagia and death in advanced stages of the disease. In order to study the molecular mechanisms leading to the degenerative loss of adult muscle tissue in DM1, we generated an inducible Drosophila model of expanded CTG trinucleotide repeat toxicity that resembles an adult-onset form of the disease. ... results were validated in skeletal muscle biopsies from DM1 patients ... and also in DM1 myoblasts ... These findings provide new insights into the signaling pathways involved in DM1 disease pathogenesis."

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