Fig. 5
From: A novel recessive mutation affecting DNAJB6a causes myofibrillar myopathy
Tibialis anterior muscle histopathology of HOM and WT mice. a–c Representative HE (d), SDH (e) and COX (f) staining of tibialis anterior (TA) muscle from 3,12,18-month-old WT and HOM mice. Scale Bar = 100 µm. d Electron microscopy images of the TA muscle of 3-month-old HOM mice showed basically normal mitochondria regularly positioned adjacent to myofibrillar Z-discs (1). 12-month-old HOM mice displayed vacuolated (white arrow, 2) and enlarged mitochondria (white asterisk, 2) in the intermyofibrillar space, focal accumulations of mitochondria in the subsarcolemmal region (ma, 3), some of them undergoing mitophagy (white arrowheads, 3). 18-month-old HOM mice displayed severe myofiber disruption (black arrowheads, 4) and vacuolated mitochondria (black asterisk, 4). Scale bars = 0.5 µm (1, 3, 4), 1.0 µm (2)