Abstract
Cynthia Machado Cascabulho* and Andrea Henriques-Pons Author Affiliations Laboratório de Inovações em Terapias, Ensino e Bioprodutos, Instituto Oswaldo Cruz; Fundação Oswaldo Cruz, Rio de Janeiro (RJ), Brasil Received: December 31, 2020 | Published: January 13, 2020 Corresponding author: Cynthia Machado Cascabulho, Instituto Oswaldo Cruz /Fundação Oswaldo Cruz, Laboratório de Inovações em Terapias, Ensino e Bioprodutos, Pavilhão Cardoso Fontes, sala 22, Rio de Janeiro (RJ), Av. Brasil 4365, Manguinhos, CEP 21045- 900, Brazil DOI: 10.26717/BJSTR.2020.24.004068
Highlights
Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophies, affecting 1 in 3,600 to 6,000 male births
We previously published that a subpopulation of about 30% of blood T lymphocyte collected from twelve-week old mdx mice is CD3+CD62L, different from age-matched control C57BL/10 and six-week old mdx mice that had less than 15% in average [11]
We observed that the CD62L downregulation was mediated by P2X7 activity and postulated that the lack of CD62L would lead to insufficient endothelial adhesion and reduced transmigration of possibly early-activated effector T lymphocyte
Summary
Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophies, affecting 1 in 3,600 to 6,000 male births. This neuromuscular disorder is caused by the absence of dystrophin [1], a protein that links intracellular F-actin to the dystrophin glycoprotein complex (DGC) [2], a multiprotein structure that connects the muscle fibers to the extracellular matrix. The lack of dystrophin destabilizes the muscle fibers during contraction cycles, leading to progressive cell damage through membrane leakage. Because of the progressive myonecrosis, inflammatory cells invade skeletal and cardiac muscles and promote secondary pathological damage [3]. Macrophage depletion leads to reduced myonecrosis, indicating macrophage-dependent cytotoxicity [5]
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