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Arquivos de Neuro-Psiquiatria

versión impresa ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.55 no.3B São Paulo set. 1997

http://dx.doi.org/10.1590/S0004-282X1997000400017 

Importância do camundongo mdx na fisiopatologia da distrofia muscular de Duchenne

 

The importance of mdx mouse in the pathophysiology of Duchenne's muscular distrophy

 

 

Sandra Lopes SeixasI; Jussara Lagrota-CândidoII; Wilson SavinoIII; Thereza Quirico-SantosIV

ILaboratório de Patologia Celular & Molecular, Departamento de Biologia Celular & Molecular, Universidade Federal Fluminense (UFF): M.Sc., Professora Assistente do Departamento de Morfologia
IIM.Sc., Professora Assistente do Departamento de Imunobiologia da UFF
IIIPh.D., Pesquisador Titular do Departamento de Imunologia da FIOCRUZ/RJ

IVPh.D., Professora Titular do Departamento de Biologia Celular & Molecular da UFF e Pesquisadora do Departamento de Imunologia da FIOCRUZ/RJ

 

 


RESUMO

O camundongo mdx desenvolve distrofia muscular recessiva ligada ao cromossoma X (locus Xp21.1) e não expressa distrofina. Embora não apresente intensa fibrose do tecido muscular e acúmulo de tecido adiposo, é considerado o modelo animal mais adequado da distrofia muscular de Duchenne. As alterações estruturais no tecido muscular associadas à mionecrose e presença do infiltrado inflamatório com predomínio de linfócitos e monócitos/macrófagos sugerem uma participação do sistema imunológico nesta miopatia. Além disso a modulação na expressão dos componentes da matriz extracelular no microambiente muscular nas várias fases da doença (início, mionecrose, regeneração) indicam um papel importante do conjuntivo no direcionamento das células inflamatórias para o foco da lesão muscular. O camundongo mdx coloca-se como um excelente modelo para o estudo dos mecanismos patogenéticos da mionecrose e regeneração na distrofia muscular de Duchenne, possibilitando inclusive o desenvolvimento de estratégias terapêuticas mais adequadas.

Palavras-chave: distrofia muscular, distrofina, mdx, modelo animal.


ABSTRACT

The mdx mouse develop an X-linked recessive muscular dystrophy (locus Xp21.1) and lack dystrophin expression. Despite showing less intense myofibrosis and scarce deposition of fatty tissue, mdx mice are considered an adequate animal model for studies on the pathogenesis of Duchenne-type muscular dystrophy. Marked histological alterations in the muscular tissues associated to myonecrosis and inflammatory mononuclear cell infiltrate (lymphocytes, monocytes/macrophages) suggest a participation of the immune system in this myopathy. Modulation of the extracellular matrix (ECM) components in the muscular tissue during all phases (onset, myonecrosis and regeneration) of disease, indicate an important role for the ECM driving inflammatory cells to the foci of lesion. Therefore mdx mice should be regarded as an important tool for studies on pathogenetic mechanisms of Duchenne-type muscular dystrophy. Such experimental model would allow development of new therapeutic approaches for increasing survival and clinical amelioration.

Key-words: Duchenne muscular dystrophy, dystrophin, mdx, animal model.


 

 

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

 

 

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Aceite: 25-abril-1997.

 

 

Dra. Thereza Quirico-Santos - Laboratório de Patologia Celular & Molecular, Departamento de Biologia Celular & Molecular, Instituto de Biologia, UFF - 24030-120 Niterói RJ - Brasil. Fax 021 719 5934.