Article
Biochemical and mechanical dysfunction in a mouse model of desmin-related myopathy.
Circulation research - 24 Apr 2009
Maloyan Alina, Osinska Hanna, Lammerding Jan, Lee Richard T, Cingolani Oscar H, Kass David A, Lorenz John N, Robbins Jeffrey
Abstract excerpt
An R120G mutation in alphaB-crystallin (CryAB(R120G)) causes desmin-related myopathy (DRM). In mice with cardiomyocyte-specific expression of the mutation, CryAB(R120G)-mediated DRM is characterized by CryAB and desmin accumulations within cardiac muscle, mitochondrial deficiencies, activation of apoptosis, and heart failure (HF). Excessive production of reactive oxygen species (ROS) is often a hallmark of HF and...
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