Characterization of amyloid deposits found in internal organs of mdx mice

Abstract

The mdx mouse strain is the most widely used experimental model of Duchenne muscular dystrophy (DMD). Although it was previously shown that muscle biopsy specimens obtained from patients with different types of muscular dystrophy contain amyloid, no available publications have analyzed the presence of amyloid aggregates in tissues of DMD patients or mdx mice. The objective of the present work was to verify whether the internal organs of mdx mice might accumulate amyloid. The study was performed in the myocardium, kidney, and liver specimens obtained from male and female mdx mice aged from 2 to 18 months. Using histochemical staining with Congo red, amyloid aggregates were detected in mouse organs studied, and their morphology and location were analyzed. Mass spectrometry data suggest that the most probable components of amyloid aggregates found in mdx mice are vitronectin and apolipoprotein A-II.