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Related Experiment Video

Updated: Jun 1, 2026

Assessing Functional Performance in the Mdx Mouse Model
10:32

Assessing Functional Performance in the Mdx Mouse Model

Published on: March 27, 2014

Inhibiting TGF-β activity improves respiratory function in mdx mice.

Carol A Nelson1, R Bridge Hunter, Lindsay A Quigley

  • 1Genzyme Corporation, Framingham, Massachusetts, USA. Carol.nelson@genzyme.com

The American Journal of Pathology
|June 7, 2011
PubMed
Summary

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TGF - β Signaling Pathway01:16

TGF - β Signaling Pathway

The TGF-β signaling pathway regulates cell growth, differentiation, adhesion, motility, and development. TGF-β ligands that induce TGF-β signaling are synthesized in their latent form. Several proteases or cell surface receptors such as integrins act upon the latent form, releasing the active ligand. There are three types of mammalian TGF-βs: (TGF-β1, TGF-β2, and TGF-β3) that bind as homodimers or heterodimers to TGF-β receptors. The TGF-β receptors are of three kinds RI, RII, and RIII. The RI...

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Therapeutic attenuation of transforming growth factor-beta (TGF-β) signaling improved respiratory and muscle function in Duchenne muscular dystrophy (DMD) mouse models. TGF-β antagonism offers a promising therapeutic strategy for DMD patients.

Area of Science:

  • Biomedical Research
  • Translational Medicine
  • Musculoskeletal Disorders

Background:

  • Respiratory dysfunction is a primary cause of mortality in Duchenne muscular dystrophy (DMD).
  • Elevated transforming growth factor-beta (TGF-β) levels are implicated in DMD pathophysiology.
  • Investigating therapeutic strategies to counteract TGF-β signaling is crucial for DMD treatment.

Purpose of the Study:

  • To evaluate the efficacy of TGF-β signaling inhibition in improving respiratory function in mdx mice, a model for DMD.
  • To compare the effects of a TGF-β neutralizing antibody (1D11) and losartan (an angiotensin receptor antagonist) on respiratory and muscle parameters.

Main Methods:

  • mdx mice were treated with 1D11, losartan, or a combination from 2 weeks to 2 or 9 months of age.

Related Experiment Videos

Last Updated: Jun 1, 2026

Assessing Functional Performance in the Mdx Mouse Model
10:32

Assessing Functional Performance in the Mdx Mouse Model

Published on: March 27, 2014

  • Respiratory function was assessed using non-invasive plethysmography.
  • Muscle strength (forelimb grip), serum creatine kinase, diaphragm muscle fiber density, and hydroxyproline levels were measured.

    • Main Results:

    • All tested treatments normalized respiratory parameters ( Penh values, breathing frequency, inspiratory flow) in mdx mice.
    • 1D11 treatment improved forelimb grip strength at both 2 and 9 months, while losartan's effect was limited to 2 months.
    • Significant improvements in serum creatine kinase and diaphragm muscle fiber density were observed across all treatment groups, with 1D11 showing superior results in reducing hydroxyproline levels.

    Conclusions:

    • Therapeutic antagonism of TGF-β signaling effectively improves respiratory and muscle function in a mouse model of DMD.
    • 1D11 demonstrated comparable or superior efficacy to losartan, suggesting TGF-β antagonism as a viable therapeutic approach for DMD.
    • Combination therapy did not provide additional benefits over 1D11 alone.