Characterization of telocytes and neuromuscular junction aspects of skeletal striated muscles from an experimental model of Duchenne muscular dystrophy

• Grant number: 22/11697-1
• Support Opportunities: Scholarships in Brazil - Doctorate
• Start date: November 01, 2023
• End date: July 31, 2026
• Field of knowledge: Biological Sciences - Morphology - Anatomy
• Principal Investigator: Adriano Polican Ciena
• Grantee: André Neri Tomiate
• Host Institution: Instituto de Biociências (IB). Universidade Estadual Paulista (UNESP). Campus de Rio Claro. Rio Claro , SP, Brazil

Abstract

Duchenne muscular dystrophy (DMD) consist in the most common of muscular dystrophies, for the comprehension of this patology are used animal models, and the mdx mouse is the most widespread. Among the consequences of the distrophy are locomotor impairment, difficulties in eating solid foods and cardiorespiratory problems. In mdx mice, skeletal striated muscles show a non-uniform degeneration-regeneration process, and as a result, they have specific characteristics. The aim of the present study is to characterize the telocytes and aspects of the neuromuscular junction in the face of structural, ultrastructural and molecular changes in the gastrocnemius muscle and tongue of mdx mice. For this study, 50 male mice will be used at 6 months of age and from two different strains: C57BL/10 (n = 25) and C57BL/10mdx (n = 25), control group and muscular dystrophy experimental model group, respectively. In both groups, light microscopy protocols will be carried out to evidence the morphological aspects, transmission electron microscopy for ultrastructural characterization and immunofluorescence, Western blot and rt-PCR to understand the molecular changes. The results may contribute to the understanding of the most common experimental model used to study DMD. The structural, molecular and ultrastructural features of tongue and m. gastrocnemius of mdx mice are essential for understanding the way degenerative and regenerative processes occur in two muscles with different functions in DMD.