English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Zendy Open

Presents the pdf analysis as premium feature

PDF Analysis

Insights

Presents the summarisation as premium feature

Summarisation

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the zaia usage as premium feature

ZAIA

Zendy Tools

Zendy Plus

Presents the access of premium content as premium feature

Premium Content

Background  Duchenne muscular dystrophy is a fatal cardiac and skeletal muscle disease resulting from mutations in the dystrophin gene. We have previously demonstrated that a dystrophin‐associated protein, sarcospan ( SSPN ), ameliorated Duchenne muscular dystrophy skeletal muscle degeneration by activating compensatory pathways that regulate muscle cell adhesion (laminin‐binding) to the extracellular matrix. Conversely, loss of  SSPN  destabilized skeletal muscle adhesion, hampered muscle regeneration, and reduced force properties. Given the importance of  SSPN  to skeletal muscle, we investigated the consequences of  SSPN  ablation in cardiac muscle and determined whether overexpression of  SSPN  into  mdx  mice ameliorates cardiac disease symptoms associated with Duchenne muscular dystrophy cardiomyopathy.    Methods and Results   SSPN ‐null mice exhibited cardiac enlargement, exacerbated cardiomyocyte hypertrophy, and increased fibrosis in response to β‐adrenergic challenge (isoproterenol; 0.8 mg/day per 2 weeks). Biochemical analysis of  SSPN ‐null cardiac muscle revealed reduced sarcolemma localization of many proteins with a known role in cardiomyopathy pathogenesis: dystrophin, the sarcoglycans (α‐, δ‐, and γ‐subunits), and β1D integrin. Transgenic overexpression of  SSPN  in Duchenne muscular dystrophy mice ( mdx   TG  ) improved cardiomyofiber cell adhesion, sarcolemma integrity, cardiac functional parameters, as well as increased expression of compensatory transmembrane proteins that mediate attachment to the extracellular matrix.    Conclusions   SSPN  regulates sarcolemmal expression of laminin‐binding complexes that are critical to cardiac muscle function and protects against transient and chronic injury, including inherited cardiomyopathy.

Sarcospan Regulates Cardiac Isoproterenol Response and Prevents Duchenne Muscular Dystrophy–Associated Cardiomyopathy