Mouse model of desmin-related cardiomyopathy.

  title={Mouse model of desmin-related cardiomyopathy.},
  author={Xuejun Wang and Hanna E Osinska and Gerald W. Dorn and Michelle L. Nieman and John N. Lorenz and Anthony Martin Gerdes and Sandra A. Witt and Thomas R. Kimball and James Gulick and Jeffrey Robbins},
  volume={103 19},
BACKGROUND The consequence of upregulation of desmin in the heart is unknown. Mutations in desmin have been linked to desmin-related myopathy (DRM), which is characterized by abnormal intrasarcoplasmic accumulation of desmin, but direct causative evidence that a desmin mutation leads to aberrant intrasarcoplasmic desmin accumulation, aggregation, and cardiomyopathy is lacking. METHODS AND RESULTS Multiple transgenic mouse lines that expressed either murine wild-type desmin or a 7-amino acid… CONTINUE READING


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