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Tipo
ArtículoDerechos de autor
Acceso restringido
Fecha del embargo
2030-01-01
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- IOC - Artigos de Periódicos [12747]
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NON-IMMUNE MECHANISMS OF MUSCLE DAMAGE IN MYOSITIS: ROLE OF THE ENDOPLASMIC RETICULUM STRESS RESPONSE AND AUTOPHAGY IN THE DISEASE PATHOGENESIS
miopatia idiopática
músculo esquelético
morte celular
autofagia
idiopathic myopathy
skeletal muscle
cell death
autophagy
NF-kB
Afiliación
Children's National Medical Center. Dept. of Genetic Medicine. Washington, DC, USA / Fundação Oswaldo Cruz. Instituto Oswaldo Cruz. Laboratório de Inovações em Terapias, Ensino e Bioprodutos. Rio de Janeiro, RJ, Brasil.l
Children's National Medical Center. Dept. of Genetic Medicine. Washington, DC, USA.
Children's National Medical Center. Dept. of Genetic Medicine. Washington, DC, USA.
Resumen en ingles
Purpose of review—Recent literature in inflammatory myopathies suggests that both immune (cell-mediated and humoral) and non-immune (endoplasmic reticulum (ER) stress and autophagy) mechanisms play a role in muscle fiber damage and dysfunction. This review describes these findings and discusses their relevance to disease pathogenesis and therapy. Recent findings—Recent data highlights the role of ER stress response especially the roles of Hexose-6-phosphate dehydrogenase and ER-anchored RING finger E3 ligase in the activation of unfolded protein response and the formation of vacuoles and inclusions in myopathies. Several studies investigated the link between inflammation and the beta amyloid associated muscle fiber degeneration and loss of muscle function. Likewise, the roles of ER stress and autophagy in skeletal muscle damage have been explored in multiple muscle diseases. Summary—Current data indicate that the ER stress, NF-kB pathway and autophagy are active in the skeletal muscle of myositis patients, and the pro-inflammatory NF-kB pathway connects the immune and non-immune pathways of muscle damage. The relative contributions of each of these pathways to muscle fiber damage are presently unclear. Therefore further defining the role of these pathways in disease pathogenesis should help to design effective therapeutic agents for these diseases.
Palabras clave en portugues
retículo endoplasmáticomiopatia idiopática
músculo esquelético
morte celular
autofagia
Palabras clave en ingles
endoplasmic reticulumidiopathic myopathy
skeletal muscle
cell death
autophagy
NF-kB
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