Voltage-Gated Ion Channel Dysfunction Precedes Cardiomyopathy Development in the Dystrophic Heart
2011

Ion Channel Dysfunction in the Dystrophic Heart

publication Evidence: high

Author Information

Author(s): Koenig Xaver, Dysek Sandra, Kimbacher Stefanie, Mike Agnes K., Cervenka Rene, Lukacs Peter, Nagl Katrin, Dang Xuan B., Todt Hannes, Bittner Reginald E., Hilber Karlheinz

Primary Institution: Medical University of Vienna

Hypothesis

Do ion channel abnormalities occur prior to cardiac pathology in dystrophic hearts?

Conclusion

Ion channel impairments precede pathology development in the dystrophic heart, suggesting they may trigger cardiomyopathy.

Supporting Evidence

  • Dystrophin-deficiency resulted in a 25% reduction in sodium current density in neonatal cardiomyocytes.
  • Calcium channel inactivation was significantly reduced in dystrophic neonatal cardiomyocytes.
  • Sodium channel impairments slowed the upstroke of the action potential in adult cardiomyocytes.
  • The QRS interval of the electrocardiogram was prolonged only in dystrophic adult mice.

Takeaway

The study found that problems with heart cell channels happen before heart disease develops in mice with muscular dystrophy.

Methodology

The study used whole cell patch clamp techniques to investigate sodium and calcium channel properties in cardiomyocytes from dystrophic mice.

Participant Demographics

Neonatal and adult dystrophic mice (mdx and mdx-utr models).

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1371/journal.pone.0020300

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