Desmin filaments and cardiac disease: establishing causality

X Wang, H Osinska, AM Gerdes, J Robbins - Journal of cardiac failure, 2002 - Elsevier
Background: Gene targeting studies indicate that desmin is not required for embryonic
myogenesis and heart formation but is essential for maintaining the structural and functional
integrity of myocytes. Desmin-null mouse hearts developed hypertrophy and dilatation.
However, the expression of desmin is very often upregulated rather than downregulated in
many cardiac diseases. Altered distribution of desmin protein has been observed in dilated
cardiomyopathy, as well as desminopathies to which a number of mutations of desmin and a …