Gomori trichrome staining

Fourteen mtDNA tRNA mutations have been associated with maternally inherited disease. Such mutations are typically associated with severe mitochondrial myopathies, characterized by ragged red skeletal muscle fibers upon Gomori trichrome staining and the accumulation of structurally abnormal mitochondria in muscle. Mutations in tRNAs exemplify the threshold effect whereby (due to replicative segragation) individuals may not exhibit clinical signs until the proportion of mutant mtDNA exceeds 80-90%. Myoclonic epilepsy and ragged red fiber (MERRF) disease, mitochondrial encephalomyo-pathy tactic acidosis (MELAS), as well as maternally inherited myopathy and cardiomyopathy (MMC) are well-characterized mitochondrial diseases. 

Figure 11.2 Muscle pathology of Gne "hGNED176VTg mice. Hematoxylin and eosin (a) shows moderate to severe variation in fiber size with minimal endomysial fibrosis, absence of necrotic or regenerating fibers, and scattered small angular and atrophic fibers (arrowheads). Scattered fibers have rimmed vacuoles (arrows). On modified Gomori trichrome stain, fibers with rimmed vacuoles (shown by arrows in a) are shown to be spaces surrounded...

---