The effects of muscular dystrophy on the craniofacial shape of Mus musculus.

Abstract

Skeletal anomalies are common in patients with muscular dystrophy, despite an absence of mutations to genes that specifically direct skeletogenesis. In order to understand these anomalies further, we examined two strains of muscular dystrophy (laminin- and merosin-deficient) relative to controls, to determine how the weakened muscle forces affected skull shape in a mouse model. Shape was characterized with geometric morphometric techniques, improving upon the limited analytical power of the standard linear measurements. Through these techniques, we document the specific types of cranial skeletal deformation produced by the two strains, each with individual shape abnormalities. The mice with merosin deficiency (with an earlier age of onset) developed skulls with more deformation, probably related to the earlier ontogenetic timing of disease onset. Future examinations of these mouse models may provide insight regarding the impact of muscular forces and the production and maintenance of craniofacial integration and modularity.