Bortezomib (PS-341) Treatment Decreases Inflammation and Partially Rescues the Expression of the Dystrophin-Glycoprotein Complex in GRMD Dogs
Figure 2
Ultrastructural analysis of connective tissue in muscles from GRMD dogs.
A: Muscle from a healthy dog. There was a narrow endomysium space and a lower deposition of connective tissue (→). B: An untreated GRMD dog (CD). The endomysium of this dog exhibits a higher deposition of connective tissue and hypercontracted fibers (HF). C and D: Treated GRMD dogs (TD) showed a lower deposition of connective tissue and endomysium, and few fibers were hypercontracted (HF). Original magnification: 3,500×. E: In muscles from healthy dogs, the mitochondria were preserved and had the same electron density as the fibers, and the mitochondrial cristae were visible. F: GRMD dogs demonstrated abnormal mitochondria, had a higher electron density, and were smaller, and the cristae were not visible. G and H: Abnormal fiber (NF) with macrophage invasion (M), complete loss of membrane integrity and myofibrillar structure showing a finely granular cytoplasm. Activated fibroblasts (F) with a prominent rough endoplasmic reticulum (→) were present in the endomysium. Original magnification: A and B, 8,900×; C, 3,500×; D, 5,600×.