Altered myofilament structure and function in dogs with Duchenne muscular dystrophy cardiomyopathy.

ABSTRACT:

Aim

Duchenne Muscular Dystrophy (DMD) is associated with progressive depressed left ventricular (LV) function. However, DMD effects on myofilament structure and function are poorly understood. Golden Retriever Muscular Dystrophy (GRMD) is a dog model of DMD recapitulating the human form of DMD.

Objective

The objective of this study is to evaluate myofilament structure and function alterations in GRMD model with spontaneous cardiac failure.

Methods and results

We have employed synchrotron X-rays diffraction to evaluate myofilament lattice spacing at various sarcomere lengths (SL) on permeabilized LV myocardium. We found a negative correlation between SL and lattice spacing in both sub-epicardium (EPI) and sub-endocardium (ENDO) LV layers in control dog hearts. In the E

SUBMITTER: Ait Mou Y 

PROVIDER: S-EPMC5800996 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature