Mechanisms of protection against myonecrosis in extraocular muscles of the mdx mice / Mecanismos de proteção a mionecrose nos musculos extra-oculares de camundongos distroficos mdx
AUTOR(ES)
Candida Luiza Tonizza de Carvalho Melo
DATA DE PUBLICAÇÃO
2008
RESUMO
Duchenne muscular dystrophy is one of the most common hereditary diseases. Abnormal calcium ion handling renders dystrophic muscle fibers more susceptible to necrosis. In the mdx mice, extraocular muscles (EOM) are protected and do not undergo myonecrosis. We investigated whether this protection is related to an increased expression of calcium-binding proteins, which may protect against the elevated calcium levels seen in dystrophic fibers. The expression of SERCA1 and calsequestrin was examined in EOM and in non-spared limb, diaphragm and sternomastoid muscles of control and mdx mice using immunofluorescence and immunoblotting. Dystrophic EOM presented a significant increase in the proteins studied, and a significant increase in β-dystroglycan expression. These proteins were reduced in the non-spared mdx muscles. The were no changes in utrophin distribution. The increase of Ca2+-handling proteins in dystrophic EOM may permit a better maintenance of calcium homeostasis, with the consequent absence of myonecrosis. The results further support the concept that abnormal Ca2+-handling is involved in dystrophinopaties.
ASSUNTO(S)
duchenne muscular dystrophy necrose distrofia muscular de duchenne musculos extra-oculares necrosis camundongo mdx mdx mice extra-ocular muscles
ACESSO AO ARTIGO
http://libdigi.unicamp.br/document/?code=000435089Documentos Relacionados
- Protection from myonecrosis, and expression of SERCA1 and calsequestrin in dystrophic laryngeal muscles
- Efeito do ácido eicosapentaenoico na necrose e inflamação dos músculos distróficos de camundongos mdx
- Detecção da neuropatia óptica distireoidiana pela quantificação dos músculos extra-oculares e da gordura orbitária na tomografia computadorizada
- Effect of doxycycline on mionecrosis and fibrosis in skeletal muscle and heart of mice mdx dystrophy
- Populações de macrófagos em músculos esqueléticos de camundongos mdx tratados com ácido eicosapentaenóico