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Transgenic overexpression of laminin 1 chain in laminin 2 chain-deficient mice rescues the disease throughout the lifespan

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Author

Summary, in English

Several approaches to treat laminin alpha 2 chain-deficient congenital muscular dystrophy (MDC1A) in mouse models have been undertaken. Most have shown promising results in young animals. However, older animals have only been characterized to some extent. Herein we analyze the lifespan of laminin alpha 2 chain deficient mice with transgenic overexpression of laminin alpha 1 chain. Further outcome measures included internalized myonuclei, heart fibrosis, grip strength, and serum creatine kinase activity. We show that laminin alpha 2-chain-deficient animals that overexpress laminin alpha 1 chain survive to up to 1.5-2 years of age. Furthermore, they displayed improved skeletal and heart muscle morphology, near-normal muscle strength, and normalized creatine kinase levels. Such an improvement of the dystrophic phenotype that persists to old age has not been previously demonstrated in mice. Our findings hold promise with regard to the efficient treatment of MDC1A patients in the future. Muscle Nerve 42: 30-37, 2010

Department/s

Publishing year

2010

Language

English

Pages

30-37

Publication/Series

Muscle and Nerve

Volume

42

Issue

1

Document type

Journal article

Publisher

John Wiley & Sons Inc.

Topic

  • Cell and Molecular Biology

Keywords

  • muscular dystrophy
  • heart
  • gene therapy
  • muscle
  • laminin

Status

Published

Research group

  • Muscle Biology

ISBN/ISSN/Other

  • ISSN: 0148-639X