Mitochondrial Dysfunction and Apoptosis Underlie the Pathogenic Process in α-B-Crystallin Desmin-Related Cardiomyopathy

@inproceedings{AlinaMaloyan2005MitochondrialDA,
  title={Mitochondrial Dysfunction and Apoptosis Underlie the Pathogenic Process in α-B-Crystallin Desmin-Related Cardiomyopathy},
  author={AlinaMaloyan and AtsushiSanbe and HannaOsinska and MargaretWestfall and DustinRobinson and Ken-ichiImahashi and ElizabethMurphy and JeffreyRobbins},
  year={2005}
}
Background— Mitochondria and sarcomeres have a well-defined architectural relation that partially depends on the integrity of the cytoskeletal network. An R120G missense mutation in the small heat shock protein α-B-crystallin (CryAB) causes desmin-related cardiomyopathy. Desmin-related cardiomyopathy is characterized by the formation of intracellular aggregates containing CryAB and desmin that are amyloid positive, and disease can be recapitulated in transgenic mice by cardiac-specific… CONTINUE READING

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