Cardiomyopathy

Desmin-related cardiomyopathy in transgenic mice: A cardiac amyloidosis

Published in Cancer Weekly, September 21st, 2004

"An R120G missense mutation in the small heat shock protein alpha-B-crystallin (CryAB^R120G) causes desmin-related cardiomyopathy (DRM). DRM is characterized by the formation of aggregates containing CryAB and desmin, and it can be recapitulated in transgenic mice by cardiac-specific expression of the mutant protein," scientists in the United States report.

In this article, we show that expression of CryAB^R120G leads to the formation of electron-dense bodies characteristic of the DRMs and identify these bodies as aggresomes, which are characteristic of the neurodegenerative diseases

A. Sanbe and colleagues of the University of...

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Source: Cancer Weekly (2004-09-21)

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