Research ArticleMuscular Dystrophy

Targeting latent TGFβ release in muscular dystrophy

See allHide authors and affiliations

Science Translational Medicine  22 Oct 2014:
Vol. 6, Issue 259, pp. 259ra144
DOI: 10.1126/scitranslmed.3010018

You are currently viewing the editor's summary.

View Full Text

Log in to view the full text

Log in through your institution

Log in through your institution

Unhinging Muscle Injury

Disruption of the muscle plasma membrane underlies muscle injury and muscular dystrophy. Latent TGFβ binding protein 4 (LTBP4) is a membrane-associated protein that sequesters latent TGFβ. Upon injury, the hinge region of LTBP4 undergoes proteolytic cleavage to release latent TGFβ. Excessive TGFβ release and activity accelerates muscular dystrophy and muscle injury. This hinge domain of LTBP4, and consequently TGFβ release, can be inhibited using antibodies that block proteolysis of LTBP4’s hinge domain. LTBP4 blocking antibodies can be applied as a biological approach to reduce muscle injury and the adverse consequences of muscular dystrophy.