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Biomarkers for Duchenne Muscular Dystrophy: study of tongue in young and old mdx mice

Grant number: 18/25598-0
Support type:Scholarships in Brazil - Scientific Initiation
Effective date (Start): April 01, 2019
Effective date (End): August 31, 2020
Field of knowledge:Biological Sciences - Morphology
Principal Investigator:Cintia Yuri Matsumura
Grantee:Marcelo dos Santos Voltani Lorena
Home Institution: Instituto de Biociências (IBB). Universidade Estadual Paulista (UNESP). Campus de Botucatu. Botucatu , SP, Brazil
Associated scholarship(s):19/20162-1 - Analysis of tongue's metabolic profile and gene therapy on the mdx mice, BE.EP.IC

Abstract

Biomarkers are cellular, structural and biochemical components that can be used as parameters to evaluate the presence and progression of a disease. Duchenne muscular dystrophy (DMD) is a progressive degenerative disease that affects skeletal and cardiac muscles leading the patient to death. In DMD, the absence of the protein dystrophin results in sarcolemmal fragility, deficiency in its repair and regeneration, and myonecrosis. The mdx mouse, an experimental model for DMD, shows the first signs of degeneration around 20 days-old and typical dystrophic features included loss of muscle fibers, increase of fibrosis and regenerated fibers at 26-month-old. The mdx tongue seems unaffected in the early stage of the disease, however, it is the second most affected muscle by fibrosis and loss of muscle fibers at 26-month-old. The metabolomics provides an integrated picture of biochemistry and shows changes in metabolites related to the progression of myonecrosis. This project aims to analyze metabolites in mdx tongue at different ages and degrees of muscle degeneration to establish biomarkers for DMD, improve the knowledge of mechanisms of muscle degeneration/regeneration and enable the development of new therapies for the disease.