Fibrogenic cytokines and extent of fibrosis in muscle of dogs with X-linked golden retriever muscular dystrophy

Research output: Contribution to journalArticle

Abstract

Fibrosis in Duchenne muscular dystrophy patients' muscle seems to be mediated by fibrogenic cytokines, particularly transforming growth factor-β1. Golden retriever muscular dystrophy is a model of Duchenne dystrophy characterised by severe myopathy and muscle fibrosis. We evaluated mRNA levels and protein distribution of transforming growth factor-β1, connective tissue growth factor and collagens in muscle of golden retriever muscular dystrophy dogs at different ages. Fibrosis occurs early in golden retriever muscular dystrophy dogs and transforming growth factor-β1 levels tend to be high up to 60 days of age (P=0.019 at 30 days), suggesting that transforming growth factor-β1 is involved in the early stages of fibrosis. We also found greater expression of connective tissue growth factor in golden retriever muscular dystrophy than control muscle (P=0.0065 at 30 days), suggesting involvement of this molecule in fibrosis progression. Our findings sustain the hypothesis that cytokines are actively involved in fibrosis in golden retriever muscular dystrophy, as it seems to be in humans, and confirm the utility of this model for investigating new therapeutic approaches for Duchenne dystrophy.

Original languageEnglish
Pages (from-to)828-835
Number of pages8
JournalNeuromuscular Disorders
Volume12
Issue number9
DOIs
Publication statusPublished - Nov 2002

Fingerprint

Muscular Dystrophies
Fibrosis
Transforming Growth Factors
Dogs
Cytokines
Muscles
Connective Tissue Growth Factor
Duchenne Muscular Dystrophy
Muscular Diseases
Collagen
Messenger RNA
Proteins

Keywords

  • Connective tissue growth factor
  • Duchenne muscular dystrophy
  • Extracellular matrix
  • Golden retriever muscular dystrophy
  • Transforming growth factor-β1

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

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title = "Fibrogenic cytokines and extent of fibrosis in muscle of dogs with X-linked golden retriever muscular dystrophy",
abstract = "Fibrosis in Duchenne muscular dystrophy patients' muscle seems to be mediated by fibrogenic cytokines, particularly transforming growth factor-β1. Golden retriever muscular dystrophy is a model of Duchenne dystrophy characterised by severe myopathy and muscle fibrosis. We evaluated mRNA levels and protein distribution of transforming growth factor-β1, connective tissue growth factor and collagens in muscle of golden retriever muscular dystrophy dogs at different ages. Fibrosis occurs early in golden retriever muscular dystrophy dogs and transforming growth factor-β1 levels tend to be high up to 60 days of age (P=0.019 at 30 days), suggesting that transforming growth factor-β1 is involved in the early stages of fibrosis. We also found greater expression of connective tissue growth factor in golden retriever muscular dystrophy than control muscle (P=0.0065 at 30 days), suggesting involvement of this molecule in fibrosis progression. Our findings sustain the hypothesis that cytokines are actively involved in fibrosis in golden retriever muscular dystrophy, as it seems to be in humans, and confirm the utility of this model for investigating new therapeutic approaches for Duchenne dystrophy.",
keywords = "Connective tissue growth factor, Duchenne muscular dystrophy, Extracellular matrix, Golden retriever muscular dystrophy, Transforming growth factor-β1",
author = "Laura Passerini and Pia Bernasconi and Fulvio Baggi and Paolo Confalonieri and Francesca Cozzi and Ferdinando Cornelio and Renato Mantegazza",
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AU - Passerini, Laura

AU - Bernasconi, Pia

AU - Baggi, Fulvio

AU - Confalonieri, Paolo

AU - Cozzi, Francesca

AU - Cornelio, Ferdinando

AU - Mantegazza, Renato

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AB - Fibrosis in Duchenne muscular dystrophy patients' muscle seems to be mediated by fibrogenic cytokines, particularly transforming growth factor-β1. Golden retriever muscular dystrophy is a model of Duchenne dystrophy characterised by severe myopathy and muscle fibrosis. We evaluated mRNA levels and protein distribution of transforming growth factor-β1, connective tissue growth factor and collagens in muscle of golden retriever muscular dystrophy dogs at different ages. Fibrosis occurs early in golden retriever muscular dystrophy dogs and transforming growth factor-β1 levels tend to be high up to 60 days of age (P=0.019 at 30 days), suggesting that transforming growth factor-β1 is involved in the early stages of fibrosis. We also found greater expression of connective tissue growth factor in golden retriever muscular dystrophy than control muscle (P=0.0065 at 30 days), suggesting involvement of this molecule in fibrosis progression. Our findings sustain the hypothesis that cytokines are actively involved in fibrosis in golden retriever muscular dystrophy, as it seems to be in humans, and confirm the utility of this model for investigating new therapeutic approaches for Duchenne dystrophy.

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